BioPAX pathway converted from "Asparagine N-linked glycosylation" in the Reactome database.Asparagine N-linked glycosylationThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent proteinThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Synthesis of substrates in N-glycan biosythesisThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Synthesis of Dolichyl-phosphateThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>LEFT-TO-RIGHT4.1.1.33MVD decarboxylates MVA5PP to IPPPMevalonate-5-pyrophosphate is decarboxylatedThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>MVA5PPMevalonate-5-pyrophosphate(R)-5-diphosphomevalonic acidReactome DB_ID: 191403cytosolGENE ONTOLOGYGO:0005829(R)-5-diphosphomevalonic acid [ChEBI:15899](R)-5-diphosphomevalonic acidChEBICHEBI:15899Reactome Database ID Release 75191403Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=191403ReactomeR-ALL-1914033Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-191403.3Reactomehttp://www.reactome.orgCOMPOUNDC01143additional informationMIMI:0361ATPAdenosine 5'-triphosphateATP(4-)Reactome DB_ID: 113592ATP(4-) [ChEBI:30616]ATP(4-)ATPatpAdenosine 5'-triphosphateChEBICHEBI:30616Reactome Database ID Release 75113592Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113592ReactomeR-ALL-1135924Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-113592.4COMPOUNDC00002CO2carbon dioxideReactome DB_ID: 113528carbon dioxide [ChEBI:16526]carbon dioxideChEBICHEBI:16526Reactome Database ID Release 75113528Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113528ReactomeR-ALL-1135283Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-113528.3COMPOUNDC00011IPPPdelta3-isopentenyl-PPisopentenyl diphosphatedelta3-isopentenyl pyrophosphateReactome DB_ID: 191362isopentenyl diphosphate [ChEBI:16584]isopentenyl diphosphateChEBICHEBI:16584Reactome Database ID Release 75191362Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=191362ReactomeR-ALL-1913623Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-191362.3COMPOUNDC00129ADPAdenosine 5'-diphosphateADP(3-)Reactome DB_ID: 29370ADP(3-) [ChEBI:456216]ADP(3-)ADP5&apos;-O-[(phosphonatooxy)phosphinato]adenosineADP trianionChEBICHEBI:456216Reactome Database ID Release 7529370Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29370ReactomeR-ALL-293704Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29370.4COMPOUNDC00008PiOrthophosphatehydrogenphosphatePhosphateInorganic PhosphateReactome DB_ID: 29372hydrogenphosphate [ChEBI:43474]hydrogenphosphatehydrogen phosphatephosphate[PO3(OH)](2-)INORGANIC PHOSPHATE GROUPHYDROGENPHOSPHATE IONHPO4(2-)[P(OH)O3](2-)ChEBICHEBI:43474Reactome Database ID Release 7529372Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29372ReactomeR-ALL-293724Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29372.4COMPOUNDC00009ACTIVATIONMVD dimerReactome DB_ID: 9948661MVDMVDE2QYJ9Reactome DB_ID: 9948659UniProt:E2QYJ9MVDCanis familiarisNCBI Taxonomy9615UniProtE2QYJ92EQUAL400EQUALReactome Database ID Release 759948659Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9948659ReactomeR-CFA-1913171Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-191317.12Reactome Database ID Release 759948661Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9948661ReactomeR-CFA-1913411Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-191341.1GENE ONTOLOGYGO:0004163gene ontology term for cellular functionMIMI:0355Same Catalyst ActivityReactome Database ID Release 759948662Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9948662Reactome Database ID Release 759948664Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9948664ReactomeR-CFA-1914141Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-191414.1Mevalonate pyrophosphate decarboxylase (MPD) decarboxylates mevalonate-5-pyrophosphate (MVA5PP) into isopentenyl pyrophosphate (IPPP) while hydrolysing ATP to ADP and orthophosphate (Toth & Huwyler 1996).8626466Pubmed1996Molecular cloning and expression of the cDNAs encoding human and yeast mevalonate pyrophosphate decarboxylaseToth, MJHuwyler, LJ Biol Chem 271:7895-8inferred from electronic annotationEVIDENCE CODEECO:0000203LEFT-TO-RIGHT2.52.5.1DHDDS:NUS1 elongates E,E-FPP with (n)IPPP to form pPPPThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>20E,E-FPPE,E-farnesyl pyrophosphate2-trans,6-trans-farnesyl diphosphateE,E-farnesyl diphosphatetrans, trans-farnesyl pyrophosphateReactome DB_ID: 44199872-trans,6-trans-farnesyl diphosphate [ChEBI:17407]2-trans,6-trans-farnesyl diphosphateChEBICHEBI:17407Reactome Database ID Release 754419987Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4419987ReactomeR-ALL-44199873Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4419987.3pPPPpolypreneol pyrophosphatepolyprenol diphosphateditrans, polycis-polyprenol pyrophosphatepolyprenyl diphosphateReactome DB_ID: 4419982integral component of cytoplasmic side of endoplasmic reticulum membraneGENE ONTOLOGYGO:0071458polyprenyl diphosphate [ChEBI:37531]polyprenyl diphosphateChEBICHEBI:37531Reactome Database ID Release 754419982Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4419982ReactomeR-ALL-44199823Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4419982.3ACTIVATIONDHDDS:NUS1Reactome DB_ID: 9993265endoplasmic reticulum membraneGENE ONTOLOGYGO:0005789DHDDSDHDDSJ9P7C8Reactome DB_ID: 9993259UniProt:J9P7C8DHDDSUniProtJ9P7C81EQUAL333EQUALReactome Database ID Release 759993259Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9993259ReactomeR-CFA-44199771Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4419977.11NUS1NUS1E2RT93Reactome DB_ID: 9993263UniProt:E2RT93NUS1UniProtE2RT931EQUAL293EQUALReactome Database ID Release 759993263Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9993263ReactomeR-CFA-68069461Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6806946.11Reactome Database ID Release 759993265Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9993265ReactomeR-CFA-68069471Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6806947.1GENE ONTOLOGYGO:0016765Reactome Database ID Release 759993266Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9993266Reactome Database ID Release 759993268Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9993268ReactomeR-CFA-44199781Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4419978.1The ER membrane-associated enzyme dehydrodolichyl diphosphate synthase (DHDDS) mediates the sequential head-to-tail cis addition of multiple isopentyl pyrophosphate (IPP) molecules to farnesyl pyrophosphate (E,E-FPP) to produce polyprenol pyrophosphate (pPPP) (Shridas et al. 2003). Dolichol in humans contain homologues ranging from 17-23 isoprene units, the most common homologues contain 19 or 20 isoprene units (Freeman et al. 1980). Dehydrodolichyl diphosphate syntase complex subunit NUS1 (NUS1, aka Nogo-B receptor NgBR) interacts with DHDDS, enhancing its stability and promoting Dol-P production (Harrison et al. 2011).<br><br>Defects in DHDDS cause retinitis pigmentosa 59 (RP59; MIM:613861), a pigment retinopathy, characterised by retinal pigment deposits (visible on fundus examination) and primary loss of rod photoreceptors followed by secondary loss of cone photoreceptors. Sufferers typically have night vision blindness and loss of mid to peripheral vision. As the condition progresses, they lose far peripheral vision and eventually central vision (Zuchner et al. 2011).21295283Pubmed2011Whole-exome sequencing links a variant in DHDDS to retinitis pigmentosaZüchner, StephanDallman, JuliaWen, RongBeecham, GaryNaj, AdamFarooq, AmjadKohli, Martin AWhitehead, Patrice LHulme, WilliamKonidari, IoannaEdwards, Yvonne J KCai, GuiqingPeter, IngaSeo, DavidBuxbaum, Joseph DHaines, Jonathan LBlanton, SusanYoung, JuanAlfonso, EduardoVance, JMLam, Byron LPericak-Vance, Margaret AAm. J. Hum. Genet. 88:201-614652022Pubmed2003Identification and characterization of a cDNA encoding a long-chain cis-isoprenyltranferase involved in dolichyl monophosphate biosynthesis in the ER of brain cellsShridas, PreethaRush, Jeffrey SWaechter, Charles JBiochem. Biophys. Res. Commun. 312:1349-5621572394Pubmed2011Nogo-B receptor is necessary for cellular dolichol biosynthesis and protein N-glycosylationHarrison, Kenneth DPark, Eon JooGao, NingguoKuo, AndrewRush, Jeffrey SWaechter, Charles JLehrman, Mark ASessa, William CEMBO J. 30:2490-5007374371Pubmed1980Analysis of dolichol in human tissues by high pressure liquid chromatographyFreeman, D JRupar, C ACarroll, K KLipids 15:191-3LEFT-TO-RIGHT1.3.1SRD5A3 reduces pPNOL to DCHOLThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>TPNHNADPHReactome DB_ID: 29364NADPH [ChEBI:16474]NADPHTPNHChEBICHEBI:16474Reactome Database ID Release 7529364Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29364ReactomeR-ALL-293642Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29364.2COMPOUNDC00005pPNOLpolyprenolditrans,polycis-polyprenolditrans, polycis-polyprenolReactome DB_ID: 4419981ditrans,polycis-polyprenol [ChEBI:67132]ditrans,polycis-polyprenolChEBICHEBI:67132Reactome Database ID Release 754419981Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4419981ReactomeR-ALL-44199813Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4419981.3DCHOLdolicholReactome DB_ID: 449221dolichol [ChEBI:16091]dolicholChEBICHEBI:16091Reactome Database ID Release 75449221Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449221ReactomeR-ALL-4492213Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449221.3TPNNADP+NADPNADP(+)Nicotinamide adenine dinucleotide phosphatebeta-Nicotinamide adenine dinucleotide phosphateTriphosphopyridine nucleotideReactome DB_ID: 29366NADP(+) [ChEBI:18009]NADP(+)ChEBICHEBI:18009Reactome Database ID Release 7529366Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29366ReactomeR-ALL-293663Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29366.3COMPOUNDC00006ACTIVATIONSRD5A3SRD5A3E2RAH3Reactome DB_ID: 9993272UniProt:E2RAH3SRD5A3UniProtE2RAH31EQUAL318EQUALReactome Database ID Release 759993272Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9993272ReactomeR-CFA-4696631Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-469663.1GENE ONTOLOGYGO:0016628Reactome Database ID Release 759993273Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9993273Reactome Database ID Release 759993275Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9993275ReactomeR-CFA-44199791Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4419979.1Polyprenol reductase (SRD5A3), resident on the endoplasmic reticulum membrane, mediates the reduction of the alpha-isoprene unit of polyprenol (pPNOL) to form dolichol (DCHOL) in a NADPH-dependent manner (Cantagrel et al. 2010). Defects in SRD5A3 cause congenital disorder of glycosylation 1q (SRD5A3-CDG, CDG1Q; MIM:612379), a neurodevelopmental disorder characterised by under-glycosylated serum glycoproteins resulting in nervous system development, psychomotor retardation, hypotonia, coagulation disorders and immunodeficiency (Cantagrel et al. 2010, Kasapkara et al. 2012). Defects in SRD5A3 can also cause Kahrizi syndrome (KHRZ; MIM:612713), a neurodevelopmental disorder characterised by mental retardation, cataracts, coloboma, kyphosis, and coarse facial features (Kahrizi et al. 2011).20700148Pubmed2011Next generation sequencing in a family with autosomal recessive Kahrizi syndrome (OMIM 612713) reveals a homozygous frameshift mutation in SRD5A3Kahrizi, KimiaHu, Cougar HaoGarshasbi, MasoudAbedini, Seyedeh SedighehGhadami, ShirinKariminejad, RoxanaUllmann, ReinhardChen, WeiRopers, H-HilgerKuss, Andreas WNajmabadi, HosseinTzschach, AndreasEur. J. Hum. Genet. 19:115-720637498Pubmed2010SRD5A3 is required for converting polyprenol to dolichol and is mutated in a congenital glycosylation disorderCantagrel, VincentLefeber, Dirk JNg, Bobby GGuan, ZiqiangSilhavy, Jennifer LBielas, Stephanie LLehle, LudwigHombauer, HansAdamowicz, MaciejSwiezewska, EwaDe Brouwer, Arjan PBlümel, PeterSykut-Cegielska, JolantaHouliston, ScottSwistun, DominikaAli, Bassam RDobyns, William BBabovic-Vuksanovic, Dusicavan Bokhoven, HansWevers, RARaetz, Christian R HFreeze, Hudson HMorava, EvaAl-Gazali, LihadhGleeson, Joseph GCell 142:203-1722240719Pubmed2012SRD5A3-CDG: a patient with a novel mutationKasapkara, C STümer, LEzgü, F SHasanoğlu, ARace, VMatthijs, GJaeken, JEur. J. Paediatr. Neurol. 16:554-6LEFT-TO-RIGHT3.6.1.43DOLPP1 dephosphorylates DOLDP to DOLPThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>H2OwaterReactome DB_ID: 29356water [ChEBI:15377]waterChEBICHEBI:15377Reactome Database ID Release 7529356Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29356ReactomeR-ALL-293565Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29356.5COMPOUNDC00001DOLDPDolichol diphosphatedolichyl diphosphateReactome DB_ID: 449744dolichyl diphosphate [ChEBI:15750]dolichyl diphosphateChEBICHEBI:15750Reactome Database ID Release 75449744Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449744ReactomeR-ALL-4497443Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449744.3DOLPDolichol phosphatedolichyl phosphateReactome DB_ID: 449299dolichyl phosphate [ChEBI:16214]dolichyl phosphateDolichol phosphateDolichyl monophosphatedolichol monophosphateChEBICHEBI:16214Reactome Database ID Release 75449299Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449299ReactomeR-ALL-4492993Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449299.3ACTIVATIONDOLPP1DOLPP1E2RQJ9Reactome DB_ID: 9965369UniProt:E2RQJ9DOLPP1UniProtE2RQJ91EQUAL238EQUALReactome Database ID Release 759965369Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965369ReactomeR-CFA-4497061Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449706.1GENE ONTOLOGYGO:0047874Reactome Database ID Release 759965370Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965370Reactome Database ID Release 759965372Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965372ReactomeR-CFA-4462001Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446200.1In the last step of the N-glycan precursor biosynthesis pathway, the mature N-glycan (Glc3Man9GlcNAc2) is removed from the dolichyl diphosphate (DOLDP) molecule upon which it has been synthesized and attached to a nascent protein. The released DOLDP molecule is de-phosphorylated by dolichyl diphosphatase 1 (DOLPP1) to dolichyl phosphate (DOLP). DOLP is thus salvaged and be used as substrate for the synthesis of another N-glycan oligosaccharide (Wedgwood & Strominger 1980).6243292Pubmed1980Enzymatic activities in cultured human lymphocytes that dephosphorylate dolichyl pyrophosphate and dolichyl phosphateWedgwood, JFStrominger, JLJ Biol Chem 255:1120-3Reactome Database ID Release 7510027797Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10027797ReactomeR-CFA-4461991Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446199.1GENE ONTOLOGYGO:0006489gene ontology term for cellular processMIMI:0359Dolichol is a polyisoprenol lipid comprised of five-carbon isoprene units linked linearly in a head-to-tail fashion. Almost all eukaryotic membranes contain dolichol and its phosphorylated form is used in the N-glycosylation of proteins where it is used as an anchor for the N-glycan sugar to the ER membrane, and as an initiation point for the synthesis. Dolichol biosynthesis occurs on the cytoplasmic face of the ER membrane, which is where N-glycosylation occurs too, so is perfectly placed to serve as a substrate for this process. Dolichyl phosphate can be obtained either from direct phosphorylation of dolichol, formed in a series of reactions from mevalonate 5-pyrophosphate, or a salvage reaction by de-phosphorylation of dolichyl diphosphate, released at the end of N-glycan biosynthesis (Cantagrel & Lefeber 2011).21384228Pubmed2011From glycosylation disorders to dolichol biosynthesis defects: a new class of metabolic diseasesCantagrel, VincentLefeber, Dirk JJ. Inherit. Metab. Dis. 34:859-67Synthesis of UDP-N-acetyl-glucosamineThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>LEFT-TO-RIGHT2.7.1.59NAGK dimer phosphorylates GlcNAc, GlcNGc to GlcNAc-6-P, GlcNGc-6-PThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Converted from EntitySet in ReactomeGlcNAc, GlcNGcReactome DB_ID: 6803785GlcNAcN-acetyl-D-glucosaminealdehydo-N-acetyl-D-glucosamineN-acetylglucosamineReactome DB_ID: 2162231aldehydo-N-acetyl-D-glucosamine [ChEBI:17411]aldehydo-N-acetyl-D-glucosamineChEBICHEBI:17411Reactome Database ID Release 752162231Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=2162231ReactomeR-ALL-21622313Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-2162231.3GlcNGcN-glycolylglucosamineN-glycoloyl-D-glucosamineReactome DB_ID: 6803765N-glycoloyl-D-glucosamine [ChEBI:27459]N-glycoloyl-D-glucosamineChEBICHEBI:27459Reactome Database ID Release 756803765Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6803765ReactomeR-ALL-68037653Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6803765.3Reactome Database ID Release 756803785Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6803785ReactomeR-ALL-68037851Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6803785.1Converted from EntitySet in ReactomeGlcNAc-6-P, GlcNGc-6-PReactome DB_ID: 6803738GlcNAc-6-PN-acetyl-D-glucosamine 6-phosphateReactome DB_ID: 6803779N-acetyl-D-glucosamine 6-phosphate [ChEBI:15784]N-acetyl-D-glucosamine 6-phosphateChEBICHEBI:15784Reactome Database ID Release 756803779Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6803779ReactomeR-ALL-68037793Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6803779.3GlcNGc-6-PN-glycolylglucosamine 6-phosphateN-glycoloyl-D-glucosamine 6-phosphateReactome DB_ID: 6803743N-glycoloyl-D-glucosamine 6-phosphate [ChEBI:88130]N-glycoloyl-D-glucosamine 6-phosphateChEBICHEBI:88130Reactome Database ID Release 756803743Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6803743ReactomeR-ALL-68037433Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6803743.3Reactome Database ID Release 756803738Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6803738ReactomeR-ALL-68037381Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6803738.1H+protonhydronReactome DB_ID: 70106hydron [ChEBI:15378]hydronChEBICHEBI:15378Reactome Database ID Release 7570106Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=70106ReactomeR-ALL-701064Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-70106.4COMPOUNDC000802ACTIVATIONNAGK dimerReactome DB_ID: 10011984NAGKNAGKJ9NW96Reactome DB_ID: 10011982UniProt:J9NW96NAGKUniProtJ9NW962EQUAL344EQUALReactome Database ID Release 7510011982Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10011982ReactomeR-CFA-68037421Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6803742.12Reactome Database ID Release 7510011984Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10011984ReactomeR-CFA-68037501Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6803750.1GENE ONTOLOGYGO:0045127Reactome Database ID Release 7510011985Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10011985Reactome Database ID Release 7510011987Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10011987ReactomeR-CFA-68037711Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6803771.1Humans are not able to catalyse the formation of N-glycolylneuraminic acid (Neu5Gc) due to an inactive CMAHP enzyme. Neu5Gc can be obtained from dietary sources and must be degraded to avoid accummulation and resultant chronic inflammation known as xenosialitis (Varki et al. 2011). Degradation of excess Neu5Gc results in the formation of two ubiquitous metabolites involved in asparagine N-linked glycosylation; glycolate and glucosamine 6-phosphate. In the Neu5Gc degradation pathway, a salvage reaction of amino sugar metabolism utilises dimeric GlcNAc kinase (NAGK) to phosphorylate N-acetylglucosamine (GlcNAc) to GlcNAc-6-phosphate and N-glycolylglucosamine (GlcNGc) to GlcNGc-6-P (Hinderlich et al. 2000, Weihofen et al. 2006).10824116Pubmed2000Molecular cloning and characterization of murine and human N-acetylglucosamine kinaseHinderlich, SBerger, MSchwarzkopf, MEffertz, KReutter, WEur. J. Biochem. 267:3301-821073341Pubmed2011Biomedical differences between human and nonhuman hominids: potential roles for uniquely human aspects of sialic acid biologyVarki, Nissi MStrobert, ElizabethDick, Edward JBenirschke, KurtVarki, AjitAnnu Rev Pathol 6:365-9317010375Pubmed2006Structures of human N-Acetylglucosamine kinase in two complexes with N-Acetylglucosamine and with ADP/glucose: insights into substrate specificity and regulationWeihofen, Wilhelm ABerger, MarkusChen, HaoSaenger, WolframHinderlich, StephanJ. Mol. Biol. 364:388-99LEFT-TO-RIGHT3.5.1.25AMDHD2 hydrolyses GlcNGc-6-P to GlcN6P and CCAThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>CCAcholatecholic acidReactome DB_ID: 193473cholic acid [ChEBI:16359]cholic acidChEBICHEBI:16359Reactome Database ID Release 75193473Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=193473ReactomeR-ALL-1934733Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-193473.3GlcN6PD-glucosamine 6-phosphatealpha-D-glucosamine 6-phosphateReactome DB_ID: 449701alpha-D-glucosamine 6-phosphate [ChEBI:15873]alpha-D-glucosamine 6-phosphateChEBICHEBI:15873Reactome Database ID Release 75449701Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449701ReactomeR-ALL-4497013Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449701.3ACTIVATIONAMDHD2E2R3Q7Reactome DB_ID: 10011991UniProt:E2R3Q7UniProtE2R3Q71EQUAL409EQUALReactome Database ID Release 7510011991Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10011991ReactomeR-CFA-68038081Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6803808.1GENE ONTOLOGYGO:0008448Reactome Database ID Release 7510011992Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10011992Reactome Database ID Release 7510011994Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10011994ReactomeR-CFA-68037891Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6803789.1Humans are not able to catalyse the formation of N-glycolylneuraminic acid (Neu5Gc) due to an inactive CMAHP enzyme. Neu5Gc can be obtained from dietary sources and must be degraded to avoid accummulation and resultant chronic inflammation known as xenosialitis (Varki et al. 2011). In the Neu5Gc degradation pathway, the putative N-acetylglucosamine-6-phosphate deacetylase (AMDHD2) is thought to irreversibly hydrolyse N-glycolylglucosamine 6-phosphate (GlcNGc-6-P), resulting in the ubiquitous metabolites glycolate (CCA) and glucosamine 6-phosphate (GlcN6P) (Bergfeld et al. 2012).22692205Pubmed2012Metabolism of vertebrate amino sugars with N-glycolyl groups: elucidating the intracellular fate of the non-human sialic acid N-glycolylneuraminic acidBergfeld, Anne KPearce, Oliver M TDiaz, Sandra LPham, ThoVarki, AjitJ. Biol. Chem. 287:28865-81LEFT-TO-RIGHT2.6.1.16GFPT1,2 transfer an amino group from L-Gln to F6P to form GlcN6PFormation of glucosamine 6-phosphate from fructose 6-phosphate and glutamineThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>L-GlnL-2-Aminoglutaramic acidL-glutamine zwitterionL-glutamineReactome DB_ID: 29472L-glutamine zwitterion [ChEBI:58359]L-glutamine zwitterion146.14450(2S)-5-amino-2-ammonio-5-oxopentanoateNC(=O)CC[C@H]([NH3+])C([O-])=OC5H10N2O3(2S)-5-amino-2-azaniumyl-5-oxopentanoateL-glutamineZDXPYRJPNDTMRX-VKHMYHEASA-NInChI=1S/C5H10N2O3/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H2,7,8)(H,9,10)/t3-/m0/s1ChEBICHEBI:58359Reactome Database ID Release 7529472Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29472ReactomeR-ALL-294724Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29472.4COMPOUNDC00064Fru(6)PD-Fructose 6-phosphateketo-D-fructose 6-phosphateD-Fructose 6-phosphoric acidNeuberg esterD-fructose 6-phosphate(2-)Reactome DB_ID: 29512beta-D-fructofuranose 6-phosphate(2-) [ChEBI:57634]beta-D-fructofuranose 6-phosphate(2-)beta-D-fructofuranose 6-phosphate dianion6-O-phosphonato-beta-D-fructofuranoseChEBICHEBI:57634Reactome Database ID Release 7529512Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29512ReactomeR-ALL-295124Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29512.4COMPOUNDC00085L-GluL-GlutamateL-glutamate(1-)L-glutamic acidL-Glutaminic acidReactome DB_ID: 29404L-glutamate(1-) [ChEBI:29985]L-glutamate(1-)L-glutamic acid, ion(1-)[NH3+][C@@H](CCC([O-])=O)C([O-])=OWHUUTDBJXJRKMK-VKHMYHEASA-MC5H8NO4146.12136(2S)-2-ammoniopentanedioatehydrogen L-glutamateL-glutamateL-glutamic acid monoanionInChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/p-1/t3-/m0/s1ChEBICHEBI:29985Reactome Database ID Release 7529404Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29404ReactomeR-ALL-294044Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29404.4COMPOUNDC00025ACTIVATIONConverted from EntitySet in ReactomeGFPT1,2Reactome DB_ID: 9965868GFPT1GFPT1F1PY49Reactome DB_ID: 9965862UniProt:F1PY49GFPT1UniProtF1PY492EQUAL699EQUALReactome Database ID Release 759965862Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965862ReactomeR-CFA-5322161Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532216.1GFPT2GFPT2F1PGJ3Reactome DB_ID: 9965866UniProt:F1PGJ3GFPT2UniProtF1PGJ32EQUAL682EQUALReactome Database ID Release 759965866Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965866ReactomeR-CFA-5322081Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532208.1Reactome Database ID Release 759965868Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965868ReactomeR-CFA-5322051Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532205.1GENE ONTOLOGYGO:0004360Reactome Database ID Release 759965869Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965869Reactome Database ID Release 759965871Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965871ReactomeR-CFA-4497151Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449715.1Glucosamine-fructose 6-phosphate aminotransferases 1 and 2 (GFPT1,2) are the first and rate-limiting enzymes in the hexosamine synthesis pathway, and thus formation of hexosamines like N-acetylglucosamine (GlcNAc). These enzymes probably play a role in limiting the availability of substrates for the N- and O- linked glycosylation of proteins (McKnight et al. 1992, Oki et al. 1999). GFPT1 and 2 are required for normal functioning of neuromuscular synaptic transmission. Defects in GFPT1 lead to altered muscle fibre morphology and impaired neuromuscular junction development (Senderek et al. 2011).10198162Pubmed1999cDNA cloning and mapping of a novel subtype of glutamine:fructose-6-phosphate amidotransferase (GFAT2) in human and mouseOki, TYamazaki, KKuromitsu, JOkada, MTanaka, IGenomics 57:227-341460020Pubmed1992Molecular cloning, cDNA sequence, and bacterial expression of human glutamine:fructose-6-phosphate amidotransferaseMcKnight, GLMudri, SLMathewes, SLTraxinger, RRMarshall, SSheppard, POO'Hara, PJJ Biol Chem 267:25208-1221310273Pubmed2011Hexosamine biosynthetic pathway mutations cause neuromuscular transmission defectSenderek, JMüller, JSDusl, MStrom, TMGuergueltcheva, VDiepolder, ILaval, SHMaxwell, SCossins, JKrause, SMuelas, NVilchez, JJColomer, JMallebrera, CJNascimento, ANafissi, SKariminejad, ANilipour, YBozorgmehr, BNajmabadi, HRodolico, CSieb, JPSteinlein, OKSchlotter, BSchoser, BKirschner, JHerrmann, RVoit, TOldfors, ALindbergh, CUrtizberea, Avon der Hagen, MHübner, APalace, JBushby, KStraub, VBeeson, DAbicht, AngelaLochmüller, HAm J Hum Genet 88:162-72LEFT-TO-RIGHT2.3.1.4Acetylation of glucosamine 6-phosphate to GlcNAc6PThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Ac-CoAAcetyl coenzyme Aacetyl-CoAReactome DB_ID: 76183acetyl-CoA [ChEBI:15351]acetyl-CoAChEBICHEBI:15351Reactome Database ID Release 7576183Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=76183ReactomeR-ALL-761833Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-76183.3COMPOUNDC00024AcGlcN6PN-acetyl-D-glucosamine 6-phosphateReactome DB_ID: 449703Reactome Database ID Release 75449703Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449703ReactomeR-ALL-4497033Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449703.3CoA-SHcoenzyme AReactome DB_ID: 76194coenzyme A [ChEBI:15346]coenzyme AChEBICHEBI:15346Reactome Database ID Release 7576194Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=76194ReactomeR-ALL-761943Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-76194.3COMPOUNDC00010ACTIVATION2xGNPNAT1Reactome DB_ID: 9965877E2RJW4GNPNAT1GNPNAT1Reactome DB_ID: 9965875UniProt:E2RJW4GNPNAT1UniProtE2RJW41EQUAL184EQUALReactome Database ID Release 759965875Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965875ReactomeR-CFA-4497091Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449709.12Reactome Database ID Release 759965877Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965877ReactomeR-CFA-7716971Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-771697.1GENE ONTOLOGYGO:0004343Reactome Database ID Release 759965878Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965878Reactome Database ID Release 759965880Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965880ReactomeR-CFA-4497341Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449734.1Cytosolic GNPNAT1 catalyzes the reaction of glucosamine 6-phosphate and acetyl-CoA to form N-acetyl-glucosamine 6-phosphate (GlcNAc6P) and CoA-SH. Structural studies indicate that the active form of the enzyme is a dimer (Wang J et al, 2008).18675810Pubmed2008Acceptor substrate binding revealed by crystal structure of human glucosamine-6-phosphate N-acetyltransferase 1Wang, JLiu, XLiang, YHLi, LFSu, XDFEBS Lett 582:2973-8LEFT-TO-RIGHT5.4.2.3Isomerization of GlcNAc6P to GlcNAc1PThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>AcGlcN1PN-Acetyl-D-glucosamine 1-phosphate2-acetamido-2-deoxy-D-glucopyranose 1-phosphateReactome DB_ID: 5322042-acetamido-2-deoxy-D-glucopyranose 1-phosphate [ChEBI:7125]2-acetamido-2-deoxy-D-glucopyranose 1-phosphateChEBICHEBI:7125Reactome Database ID Release 75532204Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=532204ReactomeR-ALL-5322043Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-532204.3ACTIVATIONPGM3PGM3E2R601Reactome DB_ID: 9965329UniProt:E2R601PGM3UniProtE2R6011EQUAL542EQUALReactome Database ID Release 759965329Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965329ReactomeR-CFA-5322171Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532217.1GENE ONTOLOGYGO:0004610Reactome Database ID Release 759965330Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965330Reactome Database ID Release 759965332Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965332ReactomeR-CFA-4461851Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446185.1Cytosolic PGM3 catalyzes the isomerization of N-acetyl-D-glucosamine 6-phosphate (GlcNAc6P) to form N-acetyl-D-glucosamine 1-phosphate (GlcNAc1P) (Pang H et al, 2002).12174217Pubmed2002Identification of human phosphoglucomutase 3 (PGM3) as N-acetylglucosamine-phosphate mutase (AGM1)Pang, HKoda, YSoejima, MKimura, HAnn Hum Genet 66:139-44LEFT-TO-RIGHT2.7.7.23GlcNAc1P is dephosphorylated to UDP-N-acetyl-glucosamineThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>UTPuridine 5'-triphosphateUridine triphosphateReactome DB_ID: 29494UTP [ChEBI:15713]UTPuridine 5'-triphosphateUridine triphosphateChEBICHEBI:15713Reactome Database ID Release 7529494Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29494ReactomeR-ALL-294942Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29494.2COMPOUNDC00075UDP-GlcNAcUDP-N-acetyl-D-glucosamineUDP-N-acetyl-alpha-D-glucosamineUDP-N-acetylglucosamineReactome DB_ID: 162756UDP-N-acetyl-alpha-D-glucosamine [ChEBI:16264]UDP-N-acetyl-alpha-D-glucosamineChEBICHEBI:16264Reactome Database ID Release 75162756Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=162756ReactomeR-ALL-1627563Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-162756.3PPipyrophosphatediphosphoric acidpyrophosphoric aciddiphosphateReactome DB_ID: 111294diphosphoric acid [ChEBI:29888]diphosphoric acidChEBICHEBI:29888Reactome Database ID Release 75111294Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=111294ReactomeR-ALL-1112943Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-111294.3COMPOUNDC00013ACTIVATION2xUAP1Reactome DB_ID: 9965398UAP1UAP1F1P7W7Reactome DB_ID: 9965396UniProt:F1P7W7UAP1UniProtF1P7W71EQUAL522EQUALReactome Database ID Release 759965396Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965396ReactomeR-CFA-35584281Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-3558428.12Reactome Database ID Release 759965398Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965398ReactomeR-CFA-35584261Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-3558426.1GENE ONTOLOGYGO:0003977Reactome Database ID Release 759965399Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965399Reactome Database ID Release 759965401Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965401ReactomeR-CFA-4462041Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446204.1Cytosolic UAP1 catalyzes the reaction of N-acetyl-D-glucosamine 1-phosphate (GlcNAc1P) and UTP to UDP-N-acetyl-D-glucosamine and pyrophosphate. Structural studies indicate that the active form of the enzyme is a dimer (Peneff C et al, 2001).11707391Pubmed2001Crystal structures of two human pyrophosphorylase isoforms in complexes with UDPGlc(Gal)NAc: role of the alternatively spliced insert in the enzyme oligomeric assembly and active site architecturePeneff, CFerrari, PaolaCharrier, VTaburet, YMonnier, CZamboni, VWinter, JHarnois, MFassy, FBourne, YEMBO J 20:6191-202Reactome Database ID Release 7510028479Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10028479ReactomeR-CFA-4462101Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446210.1GENE ONTOLOGYGO:0006048UDP-acetylglucosamine acts as a donor for the first two steps of the N-glycan precursor biosynthesis pathway, and is later used as a substrate for further modifications after the precursor has been attached to the protein. It is synthesized from fructose 6-phosphate, glutamine, acetyl-CoA, and UTP in four steps.Synthesis of GDP-mannoseThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>LEFT-TO-RIGHT5.3.1.8MPI isomerises Fru6P to Man6PFructose 6-phosphate isomerizes to Mannose 6-phosphateThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Man6PD-mannose 6-phosphateReactome DB_ID: 532562D-mannose 6-phosphate [ChEBI:17369]D-mannose 6-phosphateChEBICHEBI:17369Reactome Database ID Release 75532562Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=532562ReactomeR-ALL-5325623Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-532562.3ACTIVATIONMPIMPIF1P6B2Reactome DB_ID: 9967365UniProt:F1P6B2MPIUniProtF1P6B21EQUAL423EQUALReactome Database ID Release 759967365Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9967365ReactomeR-CFA-5325111Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532511.1GENE ONTOLOGYGO:0004476Reactome Database ID Release 759967366Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9967366Reactome Database ID Release 759967368Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9967368ReactomeR-CFA-5325491Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532549.1Mannose-6-phosphate isomerase (MPI) isomerises fructose 6-phosphate (Fru6P) to mannose 6-phosphate (Man6P) (Proudfoot et al. 1994). Defects in this gene are associated with congenital disorder of glycosylation type 1B (CDG1B). Oral administration of mannose is an efficient therapy against this defect (Schollen et al. 2000).8307007Pubmed1994Purification, cDNA cloning and heterologous expression of human phosphomannose isomeraseProudfoot, AETurcatti, GWells, TNPayton, MASmith, DJEur J Biochem 219:415-2310980531Pubmed2000Genomic organization of the human phosphomannose isomerase (MPI) gene and mutation analysis in patients with congenital disorders of glycosylation type Ib (CDG-Ib)Schollen, EDorland, Lde Koning, TJVan Diggelen, OPHuijmans, JGMarquardt, TBabovic-Vuksanovic, DPatterson, MImtiaz, FWinchester, BAdamowicz, MPronicka, EFreeze, HMatthijs, GHum Mutat 16:247-52LEFT-TO-RIGHT5.4.2.8PMM1,2 isomerise Man6P to Man1PMannose 6-phosphate isomerises to Mannose 1-phosphateThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Man1PD-mannose 1-phosphateReactome DB_ID: 532531D-mannose 1-phosphate [ChEBI:35374]D-mannose 1-phosphateChEBICHEBI:35374Reactome Database ID Release 75532531Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=532531ReactomeR-ALL-5325313Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-532531.3ACTIVATIONConverted from EntitySet in ReactomePMM1,2Reactome DB_ID: 9965382PMM1PMM1F1PKB3Reactome DB_ID: 9965376UniProt:F1PKB3PMM1UniProtF1PKB31EQUAL262EQUALReactome Database ID Release 759965376Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965376ReactomeR-CFA-5325411Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532541.1PMM2PMM2E2R832Reactome DB_ID: 9965380UniProt:E2R832PMM2UniProtE2R8321EQUAL246EQUALReactome Database ID Release 759965380Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965380ReactomeR-CFA-5325691Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532569.1Reactome Database ID Release 759965382Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965382ReactomeR-CFA-5325321Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532532.1GENE ONTOLOGYGO:0004615Reactome Database ID Release 759965383Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965383Reactome Database ID Release 759965385Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965385ReactomeR-CFA-4462011Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446201.1Phosphomannomutases 1 and 2 (PMM1 and PMM2) catalyse the isomerisation of mannose 6-phosphate (Man6P) to mannose 1-phosphate (Man1P) in the cytosol of cells (Wada & Sakamoto 1997, Matthijs et al. 1997). Mutations in the PMM2 gene are one of the causes of Jaeken syndrome. a disease of glycosylation, type CDGIa. (Matthijs et al. 1997b).9119384Pubmed1997Isolation of the human phosphomannomutase gene (PMM1) and assignment to chromosome 22q13Wada, YSakamoto, MGenomics 39:416-79070917Pubmed1997PMM (PMM1), the human homologue of SEC53 or yeast phosphomannomutase, is localized on chromosome 22q13Matthijs, GSchollen, EPirard, MBudarf, MLVan Schaftingen, EmileCassiman, JJGenomics 40:41-79140401Pubmed1997Mutations in PMM2, a phosphomannomutase gene on chromosome 16p13, in carbohydrate-deficient glycoprotein type I syndrome (Jaeken syndrome)Matthijs, GSchollen, EPardon, EVeiga-da-Cunha, MJaeken, JCassiman, JJVan Schaftingen, EmileNat Genet 16:88-92Reactome Database ID Release 7510028481Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10028481ReactomeR-CFA-4462051Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446205.1GENE ONTOLOGYGO:0009298GDP-mannose is the mannose donor for the first 5 mannose addition reactions in the N-glycan precursor synthesis, and also for the synthesis of Dolichyl-phosphate-mannose involved in other mannose transfer reactions. It is synthesized from fructose 6-phosphate and GTP in three steps.Synthesis of dolichyl-phosphate mannoseThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>LEFT-TO-RIGHT2.4.1.83dolichyl phosphate + GDP-alpha-D-mannose -> dolichyl phosphate D-mannoseThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>GDP-Manguanosine 5'-(3-(alpha-D-mannopyranosyl) dihydrogen diphosphate)GDP-alpha-D-mannoseReactome DB_ID: 162860GDP-alpha-D-mannose [ChEBI:15820]GDP-alpha-D-mannoseChEBICHEBI:15820Reactome Database ID Release 75162860Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=162860ReactomeR-ALL-1628603Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-162860.3DOLP-Mandolichyl phosphate D-mannosedolichyl D-mannosyl phosphateReactome DB_ID: 162681dolichyl D-mannosyl phosphate [ChEBI:15809]dolichyl D-mannosyl phosphateDolichyl phosphate D-mannoseChEBICHEBI:15809Reactome Database ID Release 75162681Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=162681ReactomeR-ALL-1626815Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-162681.5GDPGuanosine 5'-diphosphateGuanosine diphosphateReactome DB_ID: 29420GDP [ChEBI:17552]GDPGuanosine 5'-diphosphateGuanosine diphosphateChEBICHEBI:17552Reactome Database ID Release 7529420Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29420ReactomeR-ALL-294202Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29420.2COMPOUNDC00035ACTIVATIONDPM1:DPM2:DPM3Reactome DB_ID: 9943648DPM1DPM1J9NZX5Reactome DB_ID: 9943638UniProt:J9NZX5DPM1UniProtJ9NZX52EQUAL260EQUALReactome Database ID Release 759943638Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9943638ReactomeR-CFA-1628651Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-162865.11DPM3E2RLU1Reactome DB_ID: 9943646UniProt:E2RLU1UniProtE2RLU11EQUAL92EQUALReactome Database ID Release 759943646Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9943646ReactomeR-CFA-1628251Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-162825.11DPM2DPM2F1P9X0Reactome DB_ID: 9943642UniProt:F1P9X0DPM2UniProtF1P9X02EQUAL84EQUALReactome Database ID Release 759943642Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9943642ReactomeR-CFA-1626891Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-162689.11Reactome Database ID Release 759943648Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9943648ReactomeR-CFA-1626921Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-162692.1GENE ONTOLOGYGO:0004582Reactome Database ID Release 759943649Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9943649Reactome Database ID Release 759943651Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9943651ReactomeR-CFA-1627211Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-162721.1Cytosolic GDP-mannose reacts with dolichyl phosphate in the endoplasmic reticulum membrane to form dolichyl phosphate D-mannose (DOLPman). The reaction is catalysed by dolichyl-phosphate mannosyltransferase, a heterotrimeric protein embedded in the endoplasmic reticulum membrane. The first subunit of the heterotrimer (DPM1) appears to be the actual catalyst, and the other two subunits appear to stabilise it (Maeda et al. 2000).10835346Pubmed2000Human dolichol-phosphate-mannose synthase consists of three subunits, DPM1, DPM2 and DPM3Maeda, YTanaka, SHino, JKangawa, KKinoshita, TEMBO J 19:2475-82Reactome Database ID Release 7510027401Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10027401ReactomeR-CFA-1626991Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-162699.1GENE ONTOLOGYGO:0018279Dolichyl-phosphate-mannose (DPM, DOLPman) is the donor of mannose groups in the synthesis of the dolichyl pyrophosphate-linked precursor oligosaccharide in asparagine-linked glycosylation, in the synthesis of the glycosyl phosphatidylinositol (GPI) anchor precursor, in protein O-mannosylation and in protein C-mannosylation. Its synthesis proceeds in two steps. First, cytosolic GDP-mannose reacts with dolichyl phosphate exposed on the cytosolic face of the endoplasmic reticulum membrane to form DPM with its mannose moiety oriented toward the cytosol. The DPM molecule then flips in the endoplasmic reticulum membrane, so that its mannose moiety is in the endoplasmic reticulum lumen, accessible to the enzymes that catalyze its transfer to growing glycolipids and glycoproteins (Kinoshita and Inoue, 2000; Maeda et al, 2000).11102867Pubmed2000Dissecting and manipulating the pathway for glycosylphosphatidylinositol-anchor biosynthesisKinoshita, TInoue, NCurr Opin Chem Biol 4:632-8Synthesis of dolichyl-phosphate-glucoseThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>LEFT-TO-RIGHT3.6.1.45NUDT14 hydrolyses UDP-Glc to G1P and UMPThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>UDP-GlcUDP-glucoseUDP-D-glucoseUDPglucoseReactome DB_ID: 29410UDP-D-glucose [ChEBI:18066]UDP-D-glucoseChEBICHEBI:18066Reactome Database ID Release 7529410Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29410ReactomeR-ALL-294103Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29410.3COMPOUNDC00029UMPuridine 5'-monophosphateuridylic acidUridine monophosphate5'Uridylic acidReactome DB_ID: 109402uridine 5'-monophosphate [ChEBI:16695]uridine 5'-monophosphateChEBICHEBI:16695Reactome Database ID Release 75109402Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=109402ReactomeR-ALL-1094024Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-109402.4COMPOUNDC00105G1PD-Glucose 1-phosphateD-glucopyranose 1-phosphatealpha-D-Glucose 1-phosphateCori esterD-Glucose alpha-1-phosphateReactome DB_ID: 29548D-glucopyranose 1-phosphate [ChEBI:16077]D-glucopyranose 1-phosphateChEBICHEBI:16077Reactome Database ID Release 7529548Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29548ReactomeR-ALL-295483Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29548.3COMPOUNDC00103ACTIVATIONNUDT14NUDT14F1PRK5Reactome DB_ID: 10013571UniProt:F1PRK5NUDT14UniProtF1PRK51EQUAL222EQUALReactome Database ID Release 7510013571Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10013571ReactomeR-CFA-68092201Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6809220.1GENE ONTOLOGYGO:0008768Reactome Database ID Release 7510013572Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10013572Reactome Database ID Release 7510013574Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10013574ReactomeR-CFA-68104641Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6810464.1Uridine diphosphate glucose pyrophosphatase (NUDT14) is a member of the Nudix hydrolase family, a ubiquitously distributed group of nucleotide pyrophosphatases. NUDT14 hydrolyses UDP-glucose (UDP-Glc) to glucose 1-phosphate (G1P) and UMP. UDP-Glc is a sugar donor in many glycosylation reactions and a cytosolic pyrophosphatase such as NUDT14 could play a role in regulating gluconeogenesis (Yagi et al. 2003).12429023Pubmed2003Cloning, expression and characterization of a mammalian Nudix hydrolase-like enzyme that cleaves the pyrophosphate bond of UDP-glucoseYagi, ToshihiroBaroja-Fernández, EdurneYamamoto, RyujiMuñoz, Francisco JoséAkazawa, THong, Kyoung SuPozueta-Romero, JavierBiochem. J. 370:409-15LEFT-TO-RIGHT2.4.1.117Synthesis of dolichyl-phosphate-glucoseThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>UDPuridine 5'-diphosphateReactome DB_ID: 110096UDP [ChEBI:17659]UDPChEBICHEBI:17659Reactome Database ID Release 75110096Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=110096ReactomeR-ALL-1100962Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-110096.2COMPOUNDC00015DbGPdolichyl beta-D-glucosyl phosphateReactome DB_ID: 532214dolichyl beta-D-glucosyl phosphate [ChEBI:15812]dolichyl beta-D-glucosyl phosphateChEBICHEBI:15812Reactome Database ID Release 75532214Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=532214ReactomeR-ALL-5322143Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-532214.3ACTIVATIONALG5ALG5E2R422Reactome DB_ID: 9965425UniProt:E2R422ALG5UniProtE2R4221EQUAL324EQUALReactome Database ID Release 759965425Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965425ReactomeR-CFA-5322111Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532211.1GENE ONTOLOGYGO:0004581Reactome Database ID Release 759965426Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965426Reactome Database ID Release 759965428Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965428ReactomeR-CFA-4462141Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446214.1Dolichyl-phosphate beta-glucosyltransferase (ALG5) associated with the endoplasmic reticulum (ER) membrane catalyzes the reaction of cytosolic UDP-glucose with dolichyl phosphate exposed on the cytosolic face of the ER membrane to form Dolichyl-P-glucose with its glucose moiety oriented toward the cytosol (Imbach T et al, 1999).10359825Pubmed1999A mutation in the human ortholog of the Saccharomyces cerevisiae ALG6 gene causes carbohydrate-deficient glycoprotein syndrome type-IcImbach, TBurda, PKuhnert, PWevers, RAAebi, MBerger, EGHennet, TProc Natl Acad Sci U S A 96:6982-7Reactome Database ID Release 7510028483Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10028483ReactomeR-CFA-4809851Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-480985.1Dolichyl-phosphate-glucose functions as a donor of glucose groups in reactions including three steps of N-glycan precursor biosynthesis. Dolichyl-phosphate-glucose itself is synthesized from UDP-glucose and dolichol phosphate on the cytosolic face of the endoplasmic reticulum membrane, then flipped to the luminal surface of that membrane.Sialic acid metabolismThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>LEFT-TO-RIGHT5.1.3.14GNE hydrolyzes/epimerises UDP-GlcNAc to ManNAc and UDPGNE hydrolyzes, epimerises UDP-GlcNAc to ManNAc and UDPThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>ManNAcN-acetyl-D-mannosaminealdehydo-N-acetyl-D-mannosamineReactome DB_ID: 4085214aldehydo-N-acetyl-D-mannosamine [ChEBI:17122]aldehydo-N-acetyl-D-mannosamineChEBICHEBI:17122Reactome Database ID Release 754085214Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4085214ReactomeR-ALL-40852143Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4085214.3ACTIVATIONGNE hexamerReactome DB_ID: 9992607GNEGNEF1PLL9Reactome DB_ID: 9992605UniProt:F1PLL9GNEUniProtF1PLL91EQUAL722EQUALReactome Database ID Release 759992605Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992605ReactomeR-CFA-37819271Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-3781927.16Reactome Database ID Release 759992607Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992607ReactomeR-CFA-37819181Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-3781918.1GENE ONTOLOGYGO:0008761Reactome Database ID Release 759992608Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992608Reactome Database ID Release 759992610Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992610ReactomeR-CFA-40850211Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085021.1UDP-N-acetylglucosamine 2-epimerase, N-acetylmannosamine kinase (GNE) is a bifunctional enzyme in the cytosol that is involved in the first two critical, rate-limiting steps of sialic acid (Neu5Ac, N-acetylneuraminic acid) biosynthesis, a main constituent of glycoconjugates. Because Neu5Ac is found at terminal positions of glycoconjugates, this molecule is involved in most cell-cell or cell-extracellular matrix interactions, serving as recognition sites. Thus, Neu5Ac plays critical roles in health and disease. In the first reaction, GNE hydrolyses and epimerises UDP-N-acetylglucosamine (UDP-GlcNAc) to N-acetylmannosamine (ManNAc) (Lucka et al. 1999, Keppler et al. 1999).<br><br>There are various disorders associated with defects in the GNE gene. Defects in GNE can cause sialuria (MIM:269921), an inborn error of metabolism characterised by cytoplasmic accumulation and increased urinary excretion of Neu5Ac (Seppala et al. 1999). Mutations causing sialuria are R266W, R266Q and R263L (Seppala et al. 1999). Defects in GNE can also cause hereditary inclusion body myopathy (IBM2; MIM:600737), an autosomal recessive neuromuscular disorder characterised by adult-onset, progressive distal and proximal muscle weakness and wastage. Muscle pathology shows rimmed vacuoles and filamentous inclusions (Eisenberg et al. 2001). The common M712T mutation can cause IBM2, as well as heterozygosity with the mutation M171V (Eisenberg et al. 2001, Argov et al. 2003, Broccolini et al. 2002). Defects in GNE can also cause Nonaka myopathy (NM; MIM:605820), an early adulthood-onset muscular disorder characterised by weakness and wastage of the lower limbs and rimmed vacuoles (Nonaka et al. 1981, Eisenberg et al. 2001). Mutations causing NK include the common V572L, either homozygous or heterozygous with C303V (Tomimitsu et al. 2002, Kayashima et al. 2002) and the heterozygous M712T with A631V indicated that NK and IBM2 are allelic, if not identical, disorders (Tomimitsu et al. 2004).11528398Pubmed2001The UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase gene is mutated in recessive hereditary inclusion body myopathyEisenberg, IAvidan, NPotikha, THochner, HChen, MOlender, TBarash, MShemesh, MSadeh, MGrabov-Nardini, GShmilevich, IFriedmann, AKarpati, GBradley, W GBaumbach, LLancet, DAsher, E BBeckmann, J SArgov, ZMitrani-Rosenbaum, SNat. Genet. 29:83-710334995Pubmed1999UDP-GlcNAc 2-epimerase: a regulator of cell surface sialylationKeppler, O THinderlich, SLangner, JSchwartz-Albiez, RReutter, WPawlita, MScience 284:1372-610330343Pubmed1999Mutations in the human UDP-N-acetylglucosamine 2-epimerase gene define the disease sialuria and the allosteric site of the enzymeSeppala, RLehto, V PGahl, W AAm. J. Hum. Genet. 64:1563-97252518Pubmed1981Familial distal myopathy with rimmed vacuole and lamellar (myeloid) body formationNonaka, ISunohara, NIshiura, SSatoyoshi, EJ. Neurol. Sci. 51:141-5511916006Pubmed2002Nonaka myopathy is caused by mutations in the UDP-N-acetylglucosamine-2-epimerase/N-acetylmannosamine kinase gene (GNE)Kayashima, TomohikoMatsuo, HidenoriSatoh, AkiraOhta, TohruYoshiura, Koh-ichiroMatsumoto, NaomichiNakane, YoshibumiNiikawa, NorioKishino, TatsuyaJ. Hum. Genet. 47:77-912473780Pubmed2002An Italian family with autosomal recessive inclusion-body myopathy and mutations in the GNE geneBroccolini, APescatori, MD'Amico, ASabino, ASilvestri, GRicci, EServidei, STonali, P AMirabella, MNeurology 59:1808-910431835Pubmed1999Primary structure and expression analysis of human UDP-N-acetyl-glucosamine-2-epimerase/N-acetylmannosamine kinase, the bifunctional enzyme in neuraminic acid biosynthesisLucka, LKrause, MDanker, KReutter, WHorstkorte, RFEBS Lett. 454:341-415136692Pubmed2004Distal myopathy with rimmed vacuoles (DMRV): new GNE mutations and splice variantTomimitsu, HShimizu, JIshikawa, KOhkoshi, NKanazawa, IMizusawa, HNeurology 62:1607-1012743242Pubmed2003Hereditary inclusion body myopathy: the Middle Eastern genetic clusterArgov, ZEisenberg, IGrabov-Nardini, GSadeh, MWirguin, ISoffer, DMitrani-Rosenbaum, SNeurology 60:1519-2312177386Pubmed2002Distal myopathy with rimmed vacuoles: novel mutations in the GNE geneTomimitsu, HIshikawa, KShimizu, JOhkoshi, NKanazawa, IMizusawa, HNeurology 59:451-4LEFT-TO-RIGHT2.7.1.60GNE phosphorylates ManNAc to ManNAc-6-PThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>ManNAc-6-PN-acetyl-D-mannosamine 6-phosphatealdehydo-N-acetyl-D-mannosamine 6-phosphateReactome DB_ID: 4085225aldehydo-N-acetyl-D-mannosamine 6-phosphate [ChEBI:28273]aldehydo-N-acetyl-D-mannosamine 6-phosphateChEBICHEBI:28273Reactome Database ID Release 754085225Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4085225ReactomeR-ALL-40852253Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4085225.3ACTIVATIONGENE ONTOLOGYGO:0009384Reactome Database ID Release 759992611Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992611Reactome Database ID Release 759992613Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992613ReactomeR-CFA-40850281Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085028.1UDP N acetylglucosamine 2 epimerase, N acetylmannosamine kinase (GNE) is a bifunctional enzyme in the cytosol that is involved in the first two critical, rate limiting steps of sialic acid (Neu5Ac, N acetylneuraminic acid) biosynthesis. In the second reaction, GNE phosphorylates N-acetylmannosamine (ManNAc) to ManNAc-6-P. There are various disorders associated with defects in the GNE gene. Defects in GNE can cause sialuria (MIM:269921), an inborn error of metabolism characterised by cytoplasmic accumulation and increased urinary excretion of Neu5Ac (Seppala et al. 1999). Mutations causing sialuria are R266W, R266Q and R263L (Seppala et al. 1999). Defects in GNE can also cause hereditary inclusion body myopathy (IBM2; MIM:600737), an autosomal recessive neuromuscular disorder characterised by adult-onset, progressive distal and proximal muscle weakness and wastage. Muscle pathology shows rimmed vacuoles and filamentous inclusions (Eisenberg et al. 2001). The common M712T mutation can cause IBM2, as well as heterozygosity with the mutation M171V (Eisenberg et al. 2001, Argov et al. 2003, Broccolini et al. 2002). Defects in GNE can also cause Nonaka myopathy (NM; MIM:605820), an early adulthood-onset muscular disorder characterised by weakness and wastage of the lower limbs and rimmed vacuoles (Nonaka et al. 1981, Eisenberg et al. 2001). Mutations causing NK include the common V572L, either homozygous or heterozygous with C303V (Tomimitsu et al. 2002, Kayashima et al. 2002) and the heterozygous M712T with A631V indicated that NK and IBM2 are allelic, if not identical, disorders (Tomimitsu et al. 2004).LEFT-TO-RIGHT2.5.1.57NANS converts ManNAc-6-P to Neu5Ac-9-PThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>PEPphosphoenolpyruvatePhosphoenolpyruvic acidReactome DB_ID: 29492phosphonatoenolpyruvate [ChEBI:58702]phosphonatoenolpyruvatephosphoenolpyruvateChEBICHEBI:58702Reactome Database ID Release 7529492Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29492ReactomeR-ALL-294924Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29492.4Neu5Ac-9-PN-acetylneuraminate 9-phosphateN-acetylneuraminic acid 9-phosphateReactome DB_ID: 4085390N-acetylneuraminic acid 9-phosphate [ChEBI:27438]N-acetylneuraminic acid 9-phosphateChEBICHEBI:27438Reactome Database ID Release 754085390Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4085390ReactomeR-ALL-40853903Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4085390.3ACTIVATIONNANSNANSE2QWN4Reactome DB_ID: 9992539UniProt:E2QWN4NANSUniProtE2QWN42EQUAL359EQUALReactome Database ID Release 759992539Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992539ReactomeR-CFA-40853961Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085396.1GENE ONTOLOGYGO:0047444Reactome Database ID Release 759992540Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992540Reactome Database ID Release 759992542Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992542ReactomeR-CFA-40849761Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4084976.1Sialic acid synthase (NANS, SAS) can convert N-acetylmannosamine 6-phosphate (ManNAc-6-P) to N-acetylneuraminate 9-phosphate (Neu5Ac-9-P) (Lawrence et al. 2000). NANS shows lower activity towards ManNAc and can convert it to Neu5Ac (not shown here, Lawrence et al. 2000).10749855Pubmed2000Cloning and expression of the human N-acetylneuraminic acid phosphate synthase gene with 2-keto-3-deoxy-D-glycero- D-galacto-nononic acid biosynthetic abilityLawrence, S MHuddleston, K APitts, L RNguyen, NLee, Y CVann, W FColeman, T ABetenbaugh, M JJ. Biol. Chem. 275:17869-77LEFT-TO-RIGHT3.1.3.29NANP dephosphorylates Neu5Ac-9-P to Neu5AcThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>SANeu5AcN-acetylneuraminic acidsialic acidReactome DB_ID: 428633N-acetylneuraminic acid [ChEBI:17012]N-acetylneuraminic acidChEBICHEBI:17012Reactome Database ID Release 75428633Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=428633ReactomeR-ALL-4286333Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-428633.3ACTIVATIONNANP:Mg2+Reactome DB_ID: 9992596NANPJ9NS70Reactome DB_ID: 9992594UniProt:J9NS70UniProtJ9NS701EQUAL248EQUALReactome Database ID Release 759992594Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992594ReactomeR-CFA-40853911Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085391.11Mg2+Magnesiummagnesium(2+)Mg++Reactome DB_ID: 29926magnesium(2+) [ChEBI:18420]magnesium(2+)ChEBICHEBI:18420Reactome Database ID Release 7529926Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29926ReactomeR-ALL-299265Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29926.5COMPOUNDC003051Reactome Database ID Release 759992596Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992596ReactomeR-CFA-40853951Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085395.1GENE ONTOLOGYGO:0050124Reactome Database ID Release 759992597Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992597Reactome Database ID Release 759992599Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992599ReactomeR-CFA-40849891Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4084989.1N-acylneuraminate 9-phosphatase (NANP) dephosphorylates N-acetylneuraminate -9-phosphate (Neu5Ac-9-P) to produce N-acetylneuraminate (Neu5Ac). NANP requires Mg²⁺ as a cofactor (Maliekal et al. 2006).16237198Pubmed2006Identification of the sequence encoding N-acetylneuraminate-9-phosphate phosphataseMaliekal, PVertommen, DidierDelpierre, GhislainVan Schaftingen, EmileGlycobiology 16:165-72LEFT-TO-RIGHT2.7.7.43CMAS transfers CMP from CTP to Neu5Ac, forming CMP-Neu5AcThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>CTPcytidine 5'-triphosphateCytidine triphosphateReactome DB_ID: 29470nucleoplasmGENE ONTOLOGYGO:0005654CTP [ChEBI:17677]CTPcytidine 5'-triphosphateCytidine triphosphateChEBICHEBI:17677Reactome Database ID Release 7529470Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29470ReactomeR-ALL-294702Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29470.2COMPOUNDC00063SANeu5AcN-acetylneuraminic acidsialic acidReactome DB_ID: 4085414Reactome Database ID Release 754085414Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4085414ReactomeR-ALL-40854143Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4085414.3PPipyrophosphatediphosphoric acidpyrophosphoric aciddiphosphateReactome DB_ID: 113541Reactome Database ID Release 75113541Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113541ReactomeR-ALL-1135413Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-113541.3COMPOUNDC00013CMP-Neu5AcCMP-N-acetylneuraminateCMP-N-acetyl-beta-neuraminic acidReactome DB_ID: 4085418CMP-N-acetyl-beta-neuraminic acid [ChEBI:16556]CMP-N-acetyl-beta-neuraminic acidChEBICHEBI:16556Reactome Database ID Release 754085418Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4085418ReactomeR-ALL-40854183Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4085418.3ACTIVATIONCMAS tetramerReactome DB_ID: 9992578CMASCMASE2QYQ4Reactome DB_ID: 9992576UniProt:E2QYQ4CMASUniProtE2QYQ41EQUAL434EQUALReactome Database ID Release 759992576Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992576ReactomeR-CFA-40854171Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085417.14Reactome Database ID Release 759992578Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992578ReactomeR-CFA-40854131Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085413.1GENE ONTOLOGYGO:0008781Reactome Database ID Release 759992579Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992579Reactome Database ID Release 759992581Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992581ReactomeR-CFA-40849821Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4084982.1Cytidine Monophosphate N-Acetylneuraminic Acid Synthetase1 (CMAS) transfers cytidine 5-monophosphate from a CTP donor to N-acetylneuraminate (Neu5Ac) to form CMP-Neu5Ac. CMAS is ubiquitously expressed and localizes to the nucleus in mammalian cells. The active form of the enzyme is a homotetramer (a dimer of dimers) (Lawrence et al. 2001, Huizing 2005). CMP-Neu5Ac is the donor substrate for sialyltransferases.2005Disease mechanisms associated with mutations of the GNE geneHuizing, MDisease mechanisms associated with mutations of the GNE gene (Book): 519-527http://www.sciencedirect.com/science/article/pii/S174067650500088XDisease mechanisms associated with mutations of the GNE geneHuizing, M11602804Pubmed2001Cloning and expression of human sialic acid pathway genes to generate CMP-sialic acids in insect cellsLawrence, S MHuddleston, K ATomiya, NNguyen, NLee, Y CVann, W FColeman, T ABetenbaugh, M JGlycoconj. J. 18:205-13LEFT-TO-RIGHTSLC35A1 exchanges CMP-Neu5Ac for CMPSLC35A1 mediates the antiport of CMP-sialic acid in exchange for CMPThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>CMPcytidine 5'-monophosphatecytidylic acidCytidine-5'-monophosphateReactome DB_ID: 727810Golgi lumenGENE ONTOLOGYGO:0005796cytidine 5'-monophosphate [ChEBI:17361]cytidine 5'-monophosphateChEBICHEBI:17361Reactome Database ID Release 75727810Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=727810ReactomeR-ALL-7278104Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-727810.4COMPOUNDC00055CMP-Neu5AcCMP-N-acetylneuraminateCMP-N-acetyl-beta-neuraminic acidReactome DB_ID: 4085435Reactome Database ID Release 754085435Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4085435ReactomeR-ALL-40854353Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4085435.3CMPcytidine 5'-monophosphatecytidylic acidCytidine-5'-monophosphateReactome DB_ID: 109395Reactome Database ID Release 75109395Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=109395ReactomeR-ALL-1093954Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-109395.4COMPOUNDC00055CMP-Neu5AcCMP-sialic acidCMP-N-acetyl-beta-neuraminic acidReactome DB_ID: 727815Reactome Database ID Release 75727815Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=727815ReactomeR-ALL-7278153Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-727815.3ACTIVATIONQ8WMR9SLC35A1SLC35A1Reactome DB_ID: 9967856Golgi membraneGENE ONTOLOGYGO:0000139UniProt:Q8WMR9SLC35A1UniProtQ8WMR91EQUAL337EQUALReactome Database ID Release 759967856Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9967856ReactomeR-CFA-7278121Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-727812.1GENE ONTOLOGYGO:0005456Reactome Database ID Release 759967857Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9967857Reactome Database ID Release 759967859Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9967859ReactomeR-CFA-7278071Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-727807.1The human gene SLC35A1 encodes the CMP-sialic acid transporter which mediates the antiport of CMP-sialic acid (CMP-Neu5Ac) into the Golgi lumen in exchange for CMP (Ishida et al. 1996). Defects in SLC35A1 are the cause of congenital disorder of glycosylation type 2F (CDG2F; MIM:603585). CDGs are a family of severe inherited diseases caused by a defect in protein N-glycosylation (Martinez-Duncker et al. 2005). 15576474Pubmed2005Genetic complementation reveals a novel human congenital disorder of glycosylation of type II, due to inactivation of the Golgi CMP-sialic acid transporterMartinez-Duncker, IDupré, TPiller, VPiller, FCandelier, JJTrichet, CTchernia, GOriol, RMollicone, RBlood 105:2671-69010752Pubmed1996Molecular cloning and characterization of a novel isoform of the human UDP-galactose transporter, and of related complementary DNAs belonging to the nucleotide-sugar transporter gene familyIshida, NMiura, NYoshioka, SKawakita, MJ Biochem 120:1074-8LEFT-TO-RIGHT2.4.99.4ST3GAL1-6 transfer Neu5Ac to terminal Gal (alpha-2,3 link)This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Gal-Rgalactosyl groupalpha-D-galactosyl groupReactome DB_ID: 4086285alpha-D-galactosyl group [ChEBI:61248]alpha-D-galactosyl groupbeta-D-galactosylalpha-D-galactopyranosyl groupChEBICHEBI:61248Reactome Database ID Release 754086285Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4086285ReactomeR-ALL-40862853Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4086285.3Neu5Ac-2,3-Gal-Ralpha-N-acetylneuraminyl-(2->3)-D-galactosyl groupReactome DB_ID: 4086277alpha-N-acetylneuraminyl-(2->3)-D-galactosyl group [ChEBI:75127]alpha-N-acetylneuraminyl-(2->3)-D-galactosyl groupChEBICHEBI:75127Reactome Database ID Release 754086277Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4086277ReactomeR-ALL-40862773Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4086277.3ACTIVATIONConverted from EntitySet in ReactomeST3GAL1-6Reactome DB_ID: 9992587E2RLK1ST3GAL1ST3GAL1Reactome DB_ID: 9971685UniProt:E2RLK1ST3GAL1UniProtE2RLK11EQUAL340EQUALReactome Database ID Release 759971685Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9971685ReactomeR-CFA-9814811Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-981481.1E2RR49ST3GAL2ST3GAL2Reactome DB_ID: 9971689UniProt:E2RR49ST3GAL2UniProtE2RR491EQUAL350EQUALReactome Database ID Release 759971689Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9971689ReactomeR-CFA-9814821Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-981482.1E2RD90ST3GAL4ST3GAL4Reactome DB_ID: 9971693UniProt:E2RD90ST3GAL4UniProtE2RD901EQUAL333EQUALReactome Database ID Release 759971693Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9971693ReactomeR-CFA-9814901Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-981490.1E2R0E6ST3GAL5ST3GAL5Reactome DB_ID: 9992585UniProt:E2R0E6ST3GAL5UniProtE2R0E61EQUAL418EQUALReactome Database ID Release 759992585Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992585ReactomeR-CFA-40862681Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4086268.1E2RCV7ST3GAL6ST3GAL6Reactome DB_ID: 9983502UniProt:E2RCV7ST3GAL6UniProtE2RCV71EQUAL331EQUALReactome Database ID Release 759983502Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9983502ReactomeR-CFA-14728751Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-1472875.1Reactome Database ID Release 759992587Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992587ReactomeR-CFA-40862901Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4086290.1GENE ONTOLOGYGO:0003836Reactome Database ID Release 759992588Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992588Reactome Database ID Release 759992590Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992590ReactomeR-CFA-40849841Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4084984.1The alpha-2,3-sialyltransferases 1-6 (ST3GAL1-6) are able to transfer a sialic acid (Neu5Ac) moiety to the terminal galactosyl (Gal-R) residue of O-glycosylated proteins and some gangliosides. Neu5Ac is added to Gal-R via an alpha-2,3 linkage (Shang et al. 1999, Kim et al. 1996, Kitagawa & Paulson 1993, Basu et al. 1993, Ishii et al. 1998, Okajima et al. 1999). Once sialylated, glycoconjugates translocate to the plasma membrane by an unknown mechanism. For simplicity, Gal is shown in a generic form where R represents other sugars O-linked to proteins which can result in a large variability in glycoconjugate structures.10206952Pubmed1999Molecular cloning of a novel alpha2,3-sialyltransferase (ST3Gal VI) that sialylates type II lactosamine structures on glycoproteins and glycolipidsOkajima, TFukumoto, SMiyazaki, HIshida, HKiso, MFurukawa, KUrano, TFurukawa, KJ Biol Chem 274:11479-868920913Pubmed1996Molecular cloning and expression of human Gal beta 1,3GalNAc alpha 2,3-sialytransferase (hST3Gal II)Kim, YJKim, KSKim, SHKim, CHKo, JHChoe, ISTsuji, SLee, YCBiochem Biophys Res Commun 228:324-79822625Pubmed1998Expression cloning and functional characterization of human cDNA for ganglioside GM3 synthaseIshii, AOhta, MWatanabe, YMatsuda, KIshiyama, KSakoe, KNakamura, MInokuchi, JSanai, YSaito, MJ. Biol. Chem. 273:31652-510504389Pubmed1999Molecular cloning and expression of Galbeta1,3GalNAc alpha2, 3-sialyltransferase from human fetal liverShang, JQiu, RWang, JLiu, JZhou, RDing, HYang, SZhang, SJin, CEur J Biochem 265:580-88611500Pubmed1996Characterization of two glycolipid: alpha 2-3sialyltransferases, SAT-3 (CMP-NeuAc:nLcOse4Cer alpha 2-3sialyltransferase) and SAT-4 (CMP-NeuAc:GgOse4Cer alpha 2-3sialyltransferase), from human colon carcinoma (Colo 205) cell lineBasu, SSBasu, MLi, ZBasu, SBiochemistry 35:5166-748333853Pubmed1993Cloning and expression of human Gal beta 1,3(4)GlcNAc alpha 2,3-sialyltransferaseKitagawa, HPaulson, JCBiochem Biophys Res Commun 194:375-82LEFT-TO-RIGHT2.4.99.1ST6GAL1,2 transfer Neu5Ac to terminal Gal (alpha-2,6 link)This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Neu5Ac-2,6-Gal-Ralpha-N-acetylneuraminyl-(2->6)-D-galactosyl groupReactome DB_ID: 4086294alpha-N-acetylneuraminyl-(2->6)-D-galactosyl group [ChEBI:75129]alpha-N-acetylneuraminyl-(2->6)-D-galactosyl groupChEBICHEBI:75129Reactome Database ID Release 754086294Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4086294ReactomeR-ALL-40862943Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4086294.3ACTIVATIONConverted from EntitySet in ReactomeST6GAL1,2Reactome DB_ID: 9992621E2R2H3ST6GAL1ST6GAL1Reactome DB_ID: 9971361UniProt:E2R2H3ST6GAL1UniProtE2R2H31EQUAL406EQUALReactome Database ID Release 759971361Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9971361ReactomeR-CFA-9758991Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-975899.1F1PUV9ST6GAL2ST6GAL2Reactome DB_ID: 9992619UniProt:F1PUV9ST6GAL2UniProtF1PUV91EQUAL529EQUALReactome Database ID Release 759992619Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992619ReactomeR-CFA-9758931Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-975893.1Reactome Database ID Release 759992621Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992621ReactomeR-CFA-9759041Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-975904.1GENE ONTOLOGYGO:0003835Reactome Database ID Release 759992622Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992622Reactome Database ID Release 759992624Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992624ReactomeR-CFA-40850331Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085033.1The beta-galactoside alpha-2,6-sialyltransferases 1 and 2 (ST6GAL1,2) are able to transfer a sialic acid (Neu5Ac) moiety to the terminal galactosyl (Gal-R) residue of O-glycosylated proteins and some gangliosides. Neu5Ac is added to Gal-R via an alpha-2,6 linkage (Wu et al. 2011, Takashima et al. 2002, Krzewinski-Recchi et al. 2003). Once sialylated, glycoconjugates translocate to the plasma membrane by an unknown mechanism. For simplicity, Gal is shown in a generic form where R represents other sugars O-linked to proteins. Highest activity is observed toward oligosaccharides that have the Gal-beta-1,4-GlcNAc sequence at the non-reducing end of their carbohydrate groups.21081508Pubmed2011Universal phosphatase-coupled glycosyltransferase assayWu, Zhengliang LEthen, Cheryl MPrather, BrittanyMachacek, MirandaJiang, WeipingGlycobiology 21:727-3312603328Pubmed2003Identification and functional expression of a second human beta-galactoside alpha2,6-sialyltransferase, ST6Gal IIKrzewinski-Recchi, MAJulien, SJuliant, STeintenier-Lelièvre, MSamyn-Petit, BMontiel, MDMir, AMCerutti, MHarduin-Lepers, ADelannoy, PEur J Biochem 270:950-6112235148Pubmed2002Characterization of the second type of human beta-galactoside alpha 2,6-sialyltransferase (ST6Gal II), which sialylates Galbeta 1,4GlcNAc structures on oligosaccharides preferentially. Genomic analysis of human sialyltransferase genesTakashima, ShouTsuji, ShuichiTsujimoto, MasafumiJ. Biol. Chem. 277:45719-28LEFT-TO-RIGHT2.4.99.3ST6GALNAC1-6 transfer Neu5Ac to terminal GalNAc (alpha-2,6 link)This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>GalNAc-RN-acetylgalactosaminyl groupN-acetyl-D-galactosaminyl groupReactome DB_ID: 4086301N-acetyl-D-galactosaminyl group [ChEBI:21507]N-acetyl-D-galactosaminyl groupChEBICHEBI:21507Reactome Database ID Release 754086301Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4086301ReactomeR-ALL-40863013Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4086301.3Neu5Ac-2,6-GalNAc-R?-N-acetylneuraminyl-(2?6)-N-acetyl-?-D-galactosaminyl groupalpha-N-acetylneuraminyl-(2->6)-N-acetyl-alpha-D-galactosaminyl groupReactome DB_ID: 4086341alpha-N-acetylneuraminyl-(2->6)-N-acetyl-alpha-D-galactosaminyl group [ChEBI:62634]alpha-N-acetylneuraminyl-(2->6)-N-acetyl-alpha-D-galactosaminyl groupChEBICHEBI:62634Reactome Database ID Release 754086341Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4086341ReactomeR-ALL-40863413Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4086341.3ACTIVATIONConverted from EntitySet in ReactomeST6GALNAC1-6Reactome DB_ID: 9992569F1PDK7ST6GALNAC1ST6GALNAC1Reactome DB_ID: 9992559UniProt:F1PDK7ST6GALNAC1UniProtF1PDK71EQUAL600EQUALReactome Database ID Release 759992559Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992559ReactomeR-CFA-9818561Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-981856.1J9NYC3ST6GALNAC2ST6GALNAC2Reactome DB_ID: 9971766UniProt:J9NYC3ST6GALNAC2UniProtJ9NYC31EQUAL374EQUALReactome Database ID Release 759971766Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9971766ReactomeR-CFA-9818111Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-981811.1E2REW6ST6GALNAC3ST6GALNAC3Reactome DB_ID: 9971757UniProt:E2REW6ST6GALNAC3UniProtE2REW61EQUAL305EQUALReactome Database ID Release 759971757Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9971757ReactomeR-CFA-9818221Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-981822.1F1P9W7ST6GALNAC4ST6GALNAC4Reactome DB_ID: 9971753UniProt:F1P9W7ST6GALNAC4UniProtF1P9W71EQUAL302EQUALReactome Database ID Release 759971753Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9971753ReactomeR-CFA-9818191Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-981819.1F1P6X5ST6GALNAC5ST6GALNAC5Reactome DB_ID: 9992563UniProt:F1P6X5ST6GALNAC5UniProtF1P6X51EQUAL336EQUALReactome Database ID Release 759992563Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992563ReactomeR-CFA-9818521Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-981852.1J9PAT6ST6GALNAC6ST6GALNAC6Reactome DB_ID: 9992567UniProt:J9PAT6ST6GALNAC6UniProtJ9PAT61EQUAL333EQUALReactome Database ID Release 759992567Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992567ReactomeR-CFA-9818551Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-981855.1Reactome Database ID Release 759992569Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992569ReactomeR-CFA-40862591Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4086259.1GENE ONTOLOGYGO:0001665Reactome Database ID Release 759992570Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992570Reactome Database ID Release 759992572Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992572ReactomeR-CFA-40849801Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4084980.1The alpha-2,6-sialyltransferases 1-6 (ST6GALNAC1-6) are able to transfer a sialic acid (Neu5Ac) moiety to the terminal N-acetylgalactosaminyl (GalNAc-R) residue of O-glycosylated proteins, glycoproteins and some gangliosides. Neu5Ac is added to GalNAc-R via an alpha-2,6 linkage (Ikehara et al. 1999, Samyn-Petit et al, 2000, Harduin-Lepers et al. 2000, Tsuchida et al. 2003, Senda et al. 2007). Once sialylated, glycoconjugates translocate to the plasma membrane by an unknown mechanism. For simplicity, GalNAc-R is shown in a generic form where R represents other sugars O-linked to proteins which can result in a large variability in glycoconjugate structures.10536037Pubmed1999Cloning and expression of a human gene encoding an N-acetylgalactosamine-alpha2,6-sialyltransferase (ST6GalNAc I): a candidate for synthesis of cancer-associated sialyl-Tn antigensIkehara, YKojima, NKurosawa, NKudo, TKono, MNishihara, SIssiki, SMorozumi, KItzkowitz, STsuda, TNishimura, S ITsuji, SNarimatsu, HGlycobiology 9:1213-2417123352Pubmed2007Identification and expression of a sialyltransferase responsible for the synthesis of disialylgalactosylgloboside in normal and malignant kidney cells: downregulation of ST6GalNAc VI in renal cancersSenda, MotohiroIto, AkihiroTsuchida, AkikoHagiwara, TomokoKaneda, TsuguhiroNakamura, YokoKasama, KenjiKiso, MakotoYoshikawa, KazuhiroKatagiri, YokoOno, YoshinariOgiso, ManabuUrano, TakeshiFurukawa, KeikoOshima, ShinichiFurukawa, KoichiBiochem. J. 402:459-7012668675Pubmed2003Synthesis of disialyl Lewis a (Le(a)) structure in colon cancer cell lines by a sialyltransferase, ST6GalNAc VI, responsible for the synthesis of alpha-series gangliosidesTsuchida, AkikoOkajima, TetsuyaFurukawa, KeikoAndo, TakayukiIshida, HideharuYoshida, ArutoNakamura, YokoKannagi, ReijiKiso, MakotoFurukawa, KoichiJ. Biol. Chem. 278:22787-9410742600Pubmed2000Molecular cloning and functional expression of human ST6GalNAc II. Molecular expression in various human cultured cellsSamyn-Petit, BKrzewinski-Recchi, MASteelant, WFDelannoy, PHarduin-Lepers, ABiochim Biophys Acta 1474:201-1111062056Pubmed2000Cloning, expression and gene organization of a human Neu5Ac alpha 2-3Gal beta 1-3GalNAc alpha 2,6-sialyltransferase: hST6GalNAcIVHarduin-Lepers, AStokes, DCSteelant, WFSamyn-Petit, BKrzewinski-Recchi, MAVallejo-Ruiz, VZanetta, JPAugé, CDelannoy, PBiochem J 352:37-48LEFT-TO-RIGHT2.4.99.8ST8SIA1-6 transfer Neu5Ac to terminal Neu5Ac residuesThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Neu5Ac-RN-acetylneuraminyl groupReactome DB_ID: 4088006N-acetylneuraminyl group [ChEBI:75133]N-acetylneuraminyl groupChEBICHEBI:75133Reactome Database ID Release 754088006Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088006ReactomeR-ALL-40880063Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088006.3Neu5Ac-2,8-Neu5Ac-Ralpha-N-acetylneuraminyl-(2->8)-alpha-N-acetylneuraminyl groupReactome DB_ID: 4087982alpha-N-acetylneuraminyl-(2->8)-alpha-N-acetylneuraminyl group [ChEBI:75130]alpha-N-acetylneuraminyl-(2->8)-alpha-N-acetylneuraminyl groupChEBICHEBI:75130Reactome Database ID Release 754087982Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4087982ReactomeR-ALL-40879823Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4087982.3ACTIVATIONConverted from EntitySet in ReactomeST8SIA1-6Reactome DB_ID: 9992552F1PHX4ST8SIA1ST8SIA1Reactome DB_ID: 9992546UniProt:F1PHX4ST8SIA1UniProtF1PHX41EQUAL356EQUALReactome Database ID Release 759992546Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992546ReactomeR-CFA-40862831Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4086283.1E2QY82ST8SIA2ST8SIA2Reactome DB_ID: 9962993UniProt:E2QY82ST8SIA2UniProtE2QY821EQUAL375EQUALReactome Database ID Release 759962993Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9962993ReactomeR-CFA-4223921Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-422392.1F1P991ST8SIA3ST8SIA3Reactome DB_ID: 9973693UniProt:F1P991ST8SIA3UniProtF1P9911EQUAL380EQUALReactome Database ID Release 759973693Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9973693ReactomeR-CFA-10221241Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-1022124.1F6X6J3ST8SIA4ST8SIA4Reactome DB_ID: 9962989UniProt:F6X6J3ST8SIA4UniProtF6X6J31EQUAL359EQUALReactome Database ID Release 759962989Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9962989ReactomeR-CFA-4223901Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-422390.1F1PKV8ST8SIA5ST8SIA5Reactome DB_ID: 9992550UniProt:F1PKV8ST8SIA5UniProtF1PKV81EQUAL376EQUALReactome Database ID Release 759992550Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992550ReactomeR-CFA-40863391Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4086339.1J9P0H5ST8SIA6ST8SIA6Reactome DB_ID: 9973689UniProt:J9P0H5ST8SIA6UniProtJ9P0H51EQUAL398EQUALReactome Database ID Release 759973689Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9973689ReactomeR-CFA-10221371Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-1022137.1Reactome Database ID Release 759992552Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992552ReactomeR-CFA-40862951Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4086295.1GENE ONTOLOGYGO:0003828Reactome Database ID Release 759992553Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992553Reactome Database ID Release 759992555Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992555ReactomeR-CFA-40849781Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4084978.1The alpha-2,8-sialyltransferases 1-6 (ST8SIA1-6) are involved in the production of gangliosides, glycoproteins and glycolipids. They transfer N-acetylneuraminate (Neu5Ac) to terminal Neu5Ac-yl groups of these glycoconjugates (Nakayama et al. 1996, Scheidegger et al. 1995, Lee et al. 1998, Angata et al. 2001, Kim et al. 1997). Once sialylated, glycoconjugates translocate to the plasma membrane by an unknown mechanism. For simplicity, Neu5Ac-R is shown in a generic form where R represents other sugars O-linked to proteins which can result in a large variability in glycoconjugate structures.7559389Pubmed1995A human STX cDNA confers polysialic acid expression in mammalian cellsScheidegger, E PSternberg, L RRoth, JLowe, J BJ. Biol. Chem. 270:22685-89199191Pubmed1997Molecular cloning and expression of human alpha2,8-sialyltransferase (hST8Sia V)Kim, Y JKim, K SDo, SKim, C HKim, S KLee, Y CBiochem. Biophys. Res. Commun. 235:327-309826427Pubmed1998Cloning and expression of cDNA for a human Sia alpha 2,3Gal beta 1, 4GlcNA:alpha 2,8-sialyltransferase (hST8Sia III)Lee, Y CKim, Y JLee, K YKim, K SKim, B UKim, H NKim, C HDo, S IArch. Biochem. Biophys. 360:41-68631981Pubmed1996Expression cloning of a human GT3 synthase. GD3 AND GT3 are synthesized by a single enzymeNakayama, JFukuda, M NHirabayashi, YKanamori, ASasaki, KNishi, TFukuda, MJ. Biol. Chem. 271:3684-9111279095Pubmed2001Unique disulfide bond structures found in ST8Sia IV polysialyltransferase are required for its activityAngata, KYen, T YEl-Battari, AMacher, B AFukuda, MJ. Biol. Chem. 276:15369-77LEFT-TO-RIGHT3.2.1.18NEU3 hydrolyzes Neu5Ac from glycoconjugatesThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Converted from EntitySet in ReactomeNEU3 substratesReactome DB_ID: 4088088Neu5Ac-2,3-Gal-Ralpha-N-acetylneuraminyl-(2->3)-D-galactosyl groupReactome DB_ID: 4088086plasma membraneGENE ONTOLOGYGO:0005886Reactome Database ID Release 754088086Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088086ReactomeR-ALL-40880863Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088086.3Neu5Ac-2,8-Neu5Ac-Ralpha-N-acetylneuraminyl-(2->8)-alpha-N-acetylneuraminyl groupReactome DB_ID: 4088098Reactome Database ID Release 754088098Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088098ReactomeR-ALL-40880983Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088098.3Reactome Database ID Release 754088088Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088088ReactomeR-ALL-40880881Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088088.1Converted from EntitySet in ReactomeGal-R, Neu5Ac-RReactome DB_ID: 4088103Gal-Rgalactosyl groupalpha-D-galactosyl groupReactome DB_ID: 4088081Reactome Database ID Release 754088081Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088081ReactomeR-ALL-40880813Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088081.3Neu5Ac-RN-acetylneuraminyl groupReactome DB_ID: 4088080Reactome Database ID Release 754088080Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088080ReactomeR-ALL-40880803Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088080.3Reactome Database ID Release 754088103Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088103ReactomeR-ALL-40881031Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088103.1ACTIVATIONNEU3SLCO2B1F1PN90Reactome DB_ID: 9977908UniProt:F1PN90SLCO2B1UniProtF1PN901EQUAL428EQUALReactome Database ID Release 759977908Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9977908ReactomeR-CFA-16056441Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-1605644.1GENE ONTOLOGYGO:0004308Reactome Database ID Release 759977909Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9977909Reactome Database ID Release 759992601Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992601ReactomeR-CFA-40849941Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4084994.1Sialidases 1-4 (NEU1-4, neuraminidases, receptor-destroying enzymes, RDEs) hydrolyze sialic acids (N-acetylneuraminic acid, Neu5Ac) to produce asialo compounds, a step in the degradation process of glycoproteins and gangliosides and are expressed in a variety of cellular locations. NEU3 localizes to the plasma membrane and hydrolyses Neu5Ac especially from gangliosides with alpha2,3- or alpha2,8-linkages present in the lipid bilayer (Wada et al. 1999, Monti et al. 2000). By regulating the composition of the lipid bilayer, NEU3 has been identified as an important regulator of trans-membrane signaling (Miyagi et al. 2008).10861246Pubmed2000Identification and expression of NEU3, a novel human sialidase associated to the plasma membraneMonti, EBassi, MTPapini, NRiboni, MManzoni, MVenerando, BCroci, GPreti, ABallabio, ATettamanti, GBorsani, GBiochem J 349:343-5110405317Pubmed1999Cloning, expression, and chromosomal mapping of a human ganglioside sialidaseWada, TYoshikawa, YTokuyama, SKuwabara, MAkita, HMiyagi, TBiochem. Biophys. Res. Commun. 261:21-718632803Pubmed2008Plasma membrane-associated sialidase as a crucial regulator of transmembrane signallingMiyagi, TaekoWada, TYamaguchi, KazunoriHata, KeikoShiozaki, KazuhiroJ. Biochem. 144:279-85LEFT-TO-RIGHT3.2.1.18NEU2 hydrolyzes Neu5Ac from glycoconjugatesThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Converted from EntitySet in ReactomeglycoconjugatesReactome DB_ID: 4088202Neu5Ac-2,3-Gal-Ralpha-N-acetylneuraminyl-(2->3)-D-galactosyl groupReactome DB_ID: 4088113Reactome Database ID Release 754088113Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088113ReactomeR-ALL-40881133Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088113.3Neu5Ac-2,6-Gal-Ralpha-N-acetylneuraminyl-(2->6)-D-galactosyl groupReactome DB_ID: 4088126Reactome Database ID Release 754088126Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088126ReactomeR-ALL-40881263Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088126.3Neu5Ac-2,6-GalNAc-R?-N-acetylneuraminyl-(2?6)-N-acetyl-?-D-galactosaminyl groupalpha-N-acetylneuraminyl-(2->6)-N-acetyl-alpha-D-galactosaminyl groupReactome DB_ID: 4088104Reactome Database ID Release 754088104Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088104ReactomeR-ALL-40881043Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088104.3Neu5Ac-2,8-Neu5Ac-Ralpha-N-acetylneuraminyl-(2->8)-alpha-N-acetylneuraminyl groupReactome DB_ID: 4088112Reactome Database ID Release 754088112Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088112ReactomeR-ALL-40881123Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088112.3Reactome Database ID Release 754088202Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088202ReactomeR-ALL-40882021Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088202.1Converted from EntitySet in ReactomeGal-R,GalNAc-R,Neu5Ac-RReactome DB_ID: 4088123Gal-Rgalactosyl groupalpha-D-galactosyl groupReactome DB_ID: 4088101Reactome Database ID Release 754088101Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088101ReactomeR-ALL-40881013Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088101.3GalNAc-RN-acetylgalactosaminyl groupN-acetyl-D-galactosaminyl groupReactome DB_ID: 4088069Reactome Database ID Release 754088069Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088069ReactomeR-ALL-40880693Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088069.3Neu5Ac-RN-acetylneuraminyl groupReactome DB_ID: 4088090Reactome Database ID Release 754088090Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088090ReactomeR-ALL-40880903Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088090.3Reactome Database ID Release 754088123Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088123ReactomeR-ALL-40881231Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088123.1ACTIVATIONNEU2NEU2J9P7D3Reactome DB_ID: 9977892UniProt:J9P7D3NEU2UniProtJ9P7D31EQUAL380EQUALReactome Database ID Release 759977892Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9977892ReactomeR-CFA-16057591Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-1605759.1Reactome Database ID Release 759977893Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9977893Reactome Database ID Release 759992615Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992615ReactomeR-CFA-40850291Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085029.1Sialidases 1-4 (NEU1-4, neuraminidases, receptor-destroying enzymes, RDEs) hydrolyze sialic acids (N-acetylneuraminic acid, Neu5Ac) to produce asialo compounds, a step in the degradation process of glycoproteins and gangliosides and are expressed in a variety of cellular locations. NEU2 (cytosolic sialidase) hydrolyzes Neu5Ac from glycoconjugates with alpha2,3-, alpha2,6- or alpha2,8-linked terminal sialated residues in the cytosol (Monti et al. 1999, Chavas et al. 2005).15501818Pubmed2005Crystal structure of the human cytosolic sialidase Neu2. Evidence for the dynamic nature of substrate recognitionChavas, Leonard M GTringali, CristinaFusi, PaolaVenerando, BrunoTettamanti, GuidoKato, RMonti, EugenioWakatsuki, SJ. Biol. Chem. 280:469-7510191093Pubmed1999Cloning and characterization of NEU2, a human gene homologous to rodent soluble sialidasesMonti, EPreti, ARossi, EBallabio, ABorsani, GGenomics 57:137-43LEFT-TO-RIGHTProton-coupled sialic acid co-transportSLC17A5 cotransports Neu5Ac, H+ from lysosomal lumen to cytosolThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>SANeu5AcN-acetylneuraminic acidsialic acidReactome DB_ID: 428639lysosomal lumenGENE ONTOLOGYGO:0043202Reactome Database ID Release 75428639Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=428639ReactomeR-ALL-4286393Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-428639.3H+hydronReactome DB_ID: 428040Reactome Database ID Release 75428040Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=428040ReactomeR-ALL-4280403Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-428040.3COMPOUNDC00080ACTIVATIONE2R8R4SLC17A5SLC17A5Reactome DB_ID: 9963671lysosomal membraneGENE ONTOLOGYGO:0005765UniProt:E2R8R4SLC17A5UniProtE2R8R41EQUAL495EQUALReactome Database ID Release 759963671Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9963671ReactomeR-CFA-4286241Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-428624.1GENE ONTOLOGYGO:0015538Reactome Database ID Release 759963672Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9963672Reactome Database ID Release 759963674Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9963674ReactomeR-CFA-4285851Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-428585.1SLC17A5 encodes a lysosomal sialic acid transporter, Sialin (AST, membrane glycoprotein HP59) (Verheijen et al. 1999, Fu et al. 2001). SLC17A5 exports sialic acid (N-acetylneuraminic acid, Neu5Ac) which is derived from the degradation of glycoconjugates. This export is dependent on the proton electrochemical gradient across the lysosomal membrane. SLC17A5 is present in the pathological tumor vasculature of the lung, breast, colon, and ovary, but not in the normal vasculature, suggesting that the protein may be critical to pathological angiogenesis. Sialin is not expressed in a variety of normal tissues, but is significantly expressed in human fetal lung. Defects in SLC17A5 cause Salla disease (SD) and infantile sialic acid storage disorder (ISSD aka N-acetylneuraminic acid storage disease, NSD). These belong to the sialic acid storage disease (SASD) group and are autosomal recessive neurodegenerative disorders characterised by hypotonia, cerebellar ataxia and mental retardation in very young infants (Verheijen et al. 1999, Aula et al. 2000).10581036Pubmed1999A new gene, encoding an anion transporter, is mutated in sialic acid storage diseasesVerheijen, FWVerbeek, EAula, NBeerens, CEHavelaar, ACJoosse, MPeltonen, LeenaAula, PGaljaard, Hvan der Spek, PJMancini, GMNat Genet 23:462-511751519Pubmed2001Identification of a novel membrane protein, HP59, with therapeutic potential as a target of tumor angiogenesisFu, CBardhan, SCetateanu, NDWamil, BDWang, YYan, HPShi, ECarter, CVenkov, CYakes, FMPage, DLLloyd, RSMernaugh, RLHellerqvist, CGClin Cancer Res 7:4182-9410947946Pubmed2000The spectrum of SLC17A5-gene mutations resulting in free sialic acid-storage diseases indicates some genotype-phenotype correlationAula, NSalomäki, PTimonen, RVerheijen, FMancini, GMånsson, JEAula, PPeltonen, LeenaAm J Hum Genet 67:832-40LEFT-TO-RIGHT4.1.3.3NPL cleaves Neu5Ac,Neu5Gc to ManNAc,ManNGc and pyruvateThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Converted from EntitySet in ReactomeNeu5Ac, Neu5GcReactome DB_ID: 4088096Neu5GcN--glycolylneuraminic acidN-glycoloylneuraminateReactome DB_ID: 4088097N-glycoloylneuraminate [ChEBI:29025]N-glycoloylneuraminateChEBICHEBI:29025Reactome Database ID Release 754088097Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088097ReactomeR-ALL-40880973Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088097.3Reactome Database ID Release 754088096Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088096ReactomeR-ALL-40880961Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088096.1PYRPyruvatepyruvic acid2-Oxopropanoate2-Oxopropanoic acidPyroracemic acidReactome DB_ID: 29398pyruvate [ChEBI:15361]pyruvate2-oxopropanoate2-oxopropanoic acid, ion(1-)ChEBICHEBI:15361Reactome Database ID Release 7529398Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29398ReactomeR-ALL-293984Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29398.4COMPOUNDC00022Converted from EntitySet in ReactomeManNAc,ManNGcReactome DB_ID: 4088099ManNGcN-glycolylmannosamineN-glycolyl-D-mannosamineReactome DB_ID: 4088084N-glycolyl-D-mannosamine [ChEBI:28255]N-glycolyl-D-mannosamineChEBICHEBI:28255Reactome Database ID Release 754088084Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088084ReactomeR-ALL-40880843Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088084.3Reactome Database ID Release 754088099Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=4088099ReactomeR-ALL-40880991Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-4088099.1ACTIVATIONNPL tetramerReactome DB_ID: 9992675NPLNPLE2RAE9Reactome DB_ID: 9992673UniProt:E2RAE9NPLUniProtE2RAE91EQUAL320EQUALReactome Database ID Release 759992673Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992673ReactomeR-CFA-40880731Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4088073.14Reactome Database ID Release 759992675Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992675ReactomeR-CFA-40880831Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4088083.1GENE ONTOLOGYGO:0008747Reactome Database ID Release 759992676Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992676Reactome Database ID Release 759992678Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9992678ReactomeR-CFA-40852171Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085217.1Once in the cytosol, sialic acids are either reutilized or degraded. N-acetylneuraminate lyase (NPL) is a cytosolic, tetrameric enzyme that can cleave the major sialic acids N-acetylneuraminic acid (Neu5Ac) and N-glycolylneuraminic acid (Neu5Gc) to form N-acetylmannosamine (ManNAc) and N-glycolylmannosamine (ManNGc) respectively (Wu et al. 2005). Although humans cannot form Neu5Gc due to a non-functional CMAHP enzyme, Neu5Gc can be ingested by dietary means and must therefore be degraded to avoid accumulation of this immunoreactive sialic acid (Bergfeld et al. 2012).16147865Pubmed2005A novel splice variant of human gene NPL, mainly expressed in human liver, kidney and peripheral blood leukocyteWu, MaoqingGu, SXu, JianZou, XianqiongZheng, HuaruiJin, ZheXie, YJi, CMao, YDNA Seq. 16:137-42Reactome Database ID Release 7510028417Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10028417ReactomeR-CFA-40850011Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4085001.1Sialic acids are a family of 9 carbon alpha-keto acids that are usually present in the non reducing terminal of glycoconjuates on the cell surface of eukaryotic cells. These sialylated conjugates play important roles in cell recognition and signaling, neuronal development, cancer metastasis and bacterial or viral infection. More than 50 forms of sialic acid are found in nature, the most abundant being N-acetylneuraminic acid (Neu5Ac, N-acetylneuraminate) (Li & Chen 2012, Wickramasinghe & Medrano 2011). The steps below describe the biosynthesis, transport, utilization and degradation of Neu5Ac in humans.22526796Pubmed2012Sialic acid metabolism and sialyltransferases: natural functions and applicationsLi, YanhongChen, XiAppl. Microbiol. Biotechnol. 94:887-90521689720Pubmed2011Primer on genes encoding enzymes in sialic acid metabolism in mammalsWickramasinghe, SaumyaMedrano, Juan FBiochimie 93:1641-6GDP-fucose biosynthesisThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>LEFT-TO-RIGHT4.2.1.47GMDS dehydrates GDP-Man to GDP-DHDManThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>GDP-DHDManGDP-4-dehydro-6-deoxy-D-mannoseGDP-4-dehydro-6-deoxy-alpha-D-mannoseReactome DB_ID: 6787618GDP-4-dehydro-6-deoxy-alpha-D-mannose [ChEBI:16955]GDP-4-dehydro-6-deoxy-alpha-D-mannoseChEBICHEBI:16955Reactome Database ID Release 756787618Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6787618ReactomeR-ALL-67876183Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6787618.3ACTIVATIONGMDSGMDSE2RC02Reactome DB_ID: 10007692UniProt:E2RC02GMDSUniProtE2RC022EQUAL372EQUALReactome Database ID Release 7510007692Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007692ReactomeR-CFA-67876201Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787620.1GENE ONTOLOGYGO:0008446Reactome Database ID Release 7510007693Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007693Reactome Database ID Release 7510007695Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007695ReactomeR-CFA-67876321Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787632.1The de novo synthesis pathway for GDP-L-fucose is a two step pathway starting from GDP-mannose. In the first step, GDP-mannose (GDP-Man) is dehydrated to GDP-4-dehydro-6-deoxy-alpha-D-mannose (GDP-DHDMan) by GDP-mannose 4,6 dehydratase (GMDS) (Sullivan et al. 1998, Ohyama et al. 1998). Fucosylation is one of the most important oligosaccharide modifications in cancer and inflammation. Defects in GMDS are involved in the progression of colorectal cancer (Nakayama et al. 2013).9603974Pubmed1998Molecular cloning and expression of GDP-D-mannose-4,6-dehydratase, a key enzyme for fucose metabolism defective in Lec13 cellsOhyama, CSmith, P LAngata, KFukuda, M NLowe, J BFukuda, MJ. Biol. Chem. 273:14582-79525924Pubmed1998Molecular cloning of human GDP-mannose 4,6-dehydratase and reconstitution of GDP-fucose biosynthesis in vitroSullivan, F XKumar, RKriz, RStahl, MXu, G YRouse, JChang, X JBoodhoo, APotvin, BCumming, D AJ. Biol. Chem. 273:8193-20223922970Pubmed2013Mutation of GDP-mannose-4,6-dehydratase in colorectal cancer metastasisNakayama, KotarosumitomoMoriwaki, KentaImai, TakuShinzaki, ShinichiroKamada, YoshihiroMurata, KoheiMiyoshi, EijiPLoS ONE 8:e70298LEFT-TO-RIGHT5TSTA3 dimer epimerises GDP-DHDMan to GDP-KDGalThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>GDP-KDGalGDP-4-keto-6-deoxygalactoseGDP-4-dehydro-beta-L-fucoseReactome DB_ID: 6787646GDP-4-dehydro-beta-L-fucose [ChEBI:86476]GDP-4-dehydro-beta-L-fucoseChEBICHEBI:86476Reactome Database ID Release 756787646Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6787646ReactomeR-ALL-67876463Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6787646.3ACTIVATIONTSTA3 dimerReactome DB_ID: 10007685GFUSTSTA3E2RM30Reactome DB_ID: 10007683UniProt:E2RM30GFUSUniProtE2RM301EQUAL321EQUALReactome Database ID Release 7510007683Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007683ReactomeR-CFA-67876151Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787615.12Reactome Database ID Release 7510007685Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007685ReactomeR-CFA-67876351Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787635.1GENE ONTOLOGYGO:0016853Reactome Database ID Release 7510007686Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007686Reactome Database ID Release 7510007688Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007688ReactomeR-CFA-67876231Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787623.1The de novo synthesis pathway for GDP-L-fucose is a two step pathway starting from GDP-mannose. In the second step, GDP-4-dehydro-6-deoxy-alpha-D-mannose (GDP-DHDMan) is epimerised and reduced to GDP-L-fucose (GDP-Fuc). The cytosolic enzyme GDP-L-fucose synthase (TSTA3, aka FX) appears to have both epimerase and reductase activities and functions as a homodimer (Tonetti et al. 1996, Zhou et al. 2013). In the first stage, the hydroxyl group at C-3 and the methyl group at C-5 of the mannose ring of GDP-DHDMan are epimerised to GDP-4-keto-6-deoxygalactose (GDP-KDGal).8910301Pubmed1996Synthesis of GDP-L-fucose by the human FX proteinTonetti, MSturla, LBisso, ABenatti, UDe Flora, AJ. Biol. Chem. 271:27274-923774504Pubmed2013The crystal structure of human GDP-L-fucose synthaseZhou, HuanSun, LihuaLi, JXu, ChunyanYu, FengLiu, YahuiJi, CHe, JianhuaActa Biochim. Biophys. Sin. (Shanghai) 45:720-5LEFT-TO-RIGHT1.1.1.271TSTA3 dimer reduces GDP-KDGal to GDP-FucThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>GDP-FucGDP-L-fucoseReactome DB_ID: 742368GDP-L-fucose [ChEBI:17009]GDP-L-fucoseChEBICHEBI:17009Reactome Database ID Release 75742368Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=742368ReactomeR-ALL-7423683Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-742368.3ACTIVATIONGENE ONTOLOGYGO:0050577Reactome Database ID Release 7510007696Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007696Reactome Database ID Release 7510007698Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007698ReactomeR-CFA-67876421Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787642.1The de novo synthesis pathway for GDP-L-fucose is a two step pathway starting from GDP-mannose. In the second step, GDP-4-dehydro-6-deoxy-alpha-D-mannose (GDP-DHDMan) is epimerised and reduced to GDP-L-fucose (GDP-Fuc). The cytosolic enzyme GDP-L-fucose synthase (TSTA3, aka FX) appears to have both epimerase and reductase activities and functions as a homodimer (Tonetti et al. 1996, Zhou et al. 2013). In the second stage, 4-reductase activity of TSTA3 dimer catalyses a hydride transfer from NADPH to the keto group at C-4 of GDP-4-keto-6-deoxygalactose (GDP-KDGal), yielding GDP-fucose (GDP-Fuc) and NADP+.LEFT-TO-RIGHT5.1.3FUOM isomerises alpha-Fuc to beta-FucThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>alpha-Fucalpha-L-fucoseReactome DB_ID: 6787679alpha-L-fucose [ChEBI:42548]alpha-L-fucosealpha-L-Fucpalpha-L-FucChEBICHEBI:42548Reactome Database ID Release 756787679Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6787679ReactomeR-ALL-67876793Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6787679.3beta-Fucbeta-L-fucoseReactome DB_ID: 6787678beta-L-fucose [ChEBI:42589]beta-L-fucosebeta-L-FucChEBICHEBI:42589Reactome Database ID Release 756787678Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6787678ReactomeR-ALL-67876783Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6787678.3ACTIVATIONFUOM dimerReactome DB_ID: 10007704FUOMFUOME2RML7Reactome DB_ID: 10007702UniProt:E2RML7FUOMUniProtE2RML71EQUAL154EQUALReactome Database ID Release 7510007702Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007702ReactomeR-CFA-67876801Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787680.12Reactome Database ID Release 7510007704Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007704ReactomeR-CFA-67876821Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787682.1GENE ONTOLOGYGO:0016857Reactome Database ID Release 7510007705Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007705Reactome Database ID Release 7510007707Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007707ReactomeR-CFA-67876771Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787677.1L-Fucose (6-deoxy-L-galactose) exists in two different forms, alpha-L-fucose (29.5%) and beta-L-fucose (70.5%). The beta-form is metabolised through the salvage pathway to ultimately form GDP-fucose. Fucose mutarotase (FUOM) is involved in the anomeric conversions of monosaccharides, converting alpha-Fuc to beta-Fuc (Timson & Reece 2003).12753898Pubmed2003Identification and characterisation of human aldose 1-epimeraseTimson, David JReece, Richard JFEBS Lett. 543:21-4LEFT-TO-RIGHT2.7.1.52FUK phosphorylates beta-Fuc to Fuc1PThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Fuc1Pfucose 1-phosphatebeta-L-fucose 1-phosphateReactome DB_ID: 6787542beta-L-fucose 1-phosphate [ChEBI:12387]beta-L-fucose 1-phosphate6-deoxy-1-O-phosphono-beta-L-galactopyranoseFucopyranosyl phosphate6-Deoxy-L-galactose 1-phosphateFucose 1-phosphateChEBICHEBI:12387Reactome Database ID Release 756787542Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6787542ReactomeR-ALL-67875423Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6787542.3ACTIVATIONFUKFCSKE2RR42Reactome DB_ID: 10007676UniProt:E2RR42FCSKUniProtE2RR421EQUAL1084EQUALReactome Database ID Release 7510007676Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007676ReactomeR-CFA-67875311Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787531.1GENE ONTOLOGYGO:0050201Reactome Database ID Release 7510007677Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007677Reactome Database ID Release 7510007679Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007679ReactomeR-CFA-67875401Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787540.1In the GDP-fucose salvage pathway, beta-L-fucose (beta-Fuc) arising from the turnover of glycoproteins and glycolipids in the liver and other tissues is reutilised via a two-step pathway. The first step is the phosphorylation of L-fucose (L-Fuc) to fucose-1-phosphate (Fuc1P) by L-fucose kinase (FUK) (Hinderlich et al. 2002).12056818Pubmed2002Identification of human L-fucose kinase amino acid sequenceHinderlich, StephanBerger, MarkusBlume, AstridChen, HaoGhaderi, DariusBauer, ChristianBiochem. Biophys. Res. Commun. 294:650-4LEFT-TO-RIGHT2.7.7.30FPGT transfers guanylyl group from GTP to Fuc1P to form GDP-FucThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>GTPGuanosine 5'-triphosphateReactome DB_ID: 29438GTP [ChEBI:15996]GTPGuanosine 5'-triphosphateChEBICHEBI:15996Reactome Database ID Release 7529438Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29438ReactomeR-ALL-294383Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29438.3COMPOUNDC00044ACTIVATIONFPGTFPGTJ9NZE3Reactome DB_ID: 10007669UniProt:J9NZE3FPGTUniProtJ9NZE31EQUAL594EQUALReactome Database ID Release 7510007669Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007669ReactomeR-CFA-67875381Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787538.1GENE ONTOLOGYGO:0047341Reactome Database ID Release 7510007670Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007670Reactome Database ID Release 7510007672Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10007672ReactomeR-CFA-67875331Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787533.1Fucose-1-phosphate guanylyltransferase (FPGT) functions in a salvage pathway to reutilise L-fucose arising from the turnover of glycoproteins and glycolipids in the liver and other tissues. In the second step of the pathway, FPGT catalyses the transfer a guanylyl group from GTP to fucose-1-phosphate (Fu1P) to form GDP-L-fucose (GDP-Fuc), the high energy donor form used by fucosyltransferases in the Golgi to add fucose moieties to growing glycan chains (Quirk & Seley 2005).16086588Pubmed2005Substrate discrimination by the human GTP fucose pyrophosphorylaseQuirk, StephenSeley, Katherine LBiochemistry 44:10854-63LEFT-TO-RIGHTSLC35C1 transport UDP-Fuc from cytosol to Golgi lumenThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>GDP-FucGDP-L-fucoseReactome DB_ID: 205683Reactome Database ID Release 75205683Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=205683ReactomeR-ALL-2056833Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-205683.3ACTIVATIONJ9NWS1SLC35C1SLC35C1Reactome DB_ID: 9967899UniProt:J9NWS1SLC35C1UniProtJ9NWS11EQUAL364EQUALReactome Database ID Release 759967899Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9967899ReactomeR-CFA-7423521Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-742352.1GENE ONTOLOGYGO:0005457Reactome Database ID Release 759967900Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9967900Reactome Database ID Release 759967902Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9967902ReactomeR-CFA-7423451Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-742345.1The human gene SLC35C1 encodes the GDP-fucose transporter FUCT1. It resides on the Golgi membrane and mediates the transport of GDP-fucose (GDP-Fuc) formed from a de novo pathway and/or a salvage pathway into the Golgi lumen. Defects in SLC35C1 causes the congenital disorder of glycosylation type 2C, also known as leukocyte adhesion deficiency type II (LAD2) (Lubke et al. 2001).11326280Pubmed2001Complementation cloning identifies CDG-IIc, a new type of congenital disorders of glycosylation, as a GDP-fucose transporter deficiencyLübke, TMarquardt, TEtzioni, AHartmann, Evon Figura, KKörner, CNat Genet 28:73-6Reactome Database ID Release 7510028573Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10028573ReactomeR-CFA-67876391Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6787639.1Fucose-containing glycans play important roles in immunity and signalling. Fucosylated glycans are created by fucosyltransferases, which require the high-energy donor substrate GDP-fucose. Two pathways for the synthesis of GDP-fucose exist in mammalian cells; the GDP-mannose-dependent de novo pathway provides the majority of GDP-fucose whereas a minor contribution comes from the free fucose-dependent salvage pathway (Becker & Lowe 2003).12651883Pubmed2003Fucose: biosynthesis and biological function in mammalsBecker, Daniel JLowe, John BGlycobiology 13:41R-53RReactome Database ID Release 7510027403Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10027403ReactomeR-CFA-4462191Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446219.1Reactions for the synthesis of the small nucleotide-linked sugar substrates that are used in the synthesis of the N-glycan precursor and in the later steps of glycosylation are annotated here.<br>All these nucleotide-linked sugar donors are synthesized in the cytosol; however, to participate in the later reactions of N-glycan precursor biosynthesis (when the glycan is oriented toward the lumen of the endoplasmic reticulum (ER)), these substrates must be attached to a dolichyl-phosphate molecule and then flipped toward the luminal side of the ER, through a mechanism which is still not known but which involves a different protein than the one that mediates the flipping of the LLO itself (Sanyal et al. 2008). Two of the genes encoding enzymes involved in these reactions, MPI and PMM2, are known to be associated with Congenital Disorders of Glycosylation (CDG) diseases of type I. Of these, CDG-Ia, associated with defects in PMM2, is the most frequent CDG disease reported.18597486Pubmed2008Distinct flippases translocate glycerophospholipids and oligosaccharide diphosphate dolichols across the endoplasmic reticulumSanyal, SFrank, CGMenon, AKBiochemistry 47:7937-46LEFT-TO-RIGHT2.7.8.15Addition of N-acetyl-D-glucosamine to Dolichyl phosphateThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>GlcNAcDOLDPN-acetyl-D-glucosaminyl-diphosphodolicholN-acetyl-D-glucosaminyldiphosphodolicholReactome DB_ID: 449222N-acetyl-D-glucosaminyldiphosphodolichol [ChEBI:18278]N-acetyl-D-glucosaminyldiphosphodolicholChEBICHEBI:18278Reactome Database ID Release 75449222Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449222ReactomeR-ALL-4492223Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449222.3ACTIVATIONDPAGT1DPAGT1E2RQ47Reactome DB_ID: 9965355UniProt:E2RQ47DPAGT1UniProtE2RQ471EQUAL408EQUALReactome Database ID Release 759965355Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965355ReactomeR-CFA-4492621Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449262.1GENE ONTOLOGYGO:0003975Reactome Database ID Release 759965356Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965356Reactome Database ID Release 759965358Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965358ReactomeR-CFA-4461911Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446191.1In the first step of N glycan precursor (LLO) synthesis, N acetylglucosamine is added, via an alpha 1,3 linkage, to a molecule of dolichyl phosphate, producing N acetyl D glucosaminyl diphosphodolichol (Eckert et al. 1998). This reaction is catalyzed by DPAGT1 (ALG7 in yeast), mutations in which are associated with CDG disorder type I J (Wu et al. 2003) and with congenital myasthenic syndrome with tubular aggregates type 2 (Belaya et al. 2012). The dolichyl phosphate acts as an anchor for the LLO, so that subsequent sugar addition reactions take place on a sugar anchored in the ER membrane. 9451016Pubmed1998Cloning and functional expression of the human GlcNAc-1-P transferase, the enzyme for the committed step of the dolichol cycle, by heterologous complementation in Saccharomyces cerevisiaeEckert, VBlank, MMazhari-Tabrizi, RMumberg, DFunk, MSchwarz, RTGlycobiology 8:77-8512872255Pubmed2003Deficiency of UDP-GlcNAc:Dolichol Phosphate N-Acetylglucosamine-1 Phosphate Transferase (DPAGT1) causes a novel congenital disorder of Glycosylation Type IjWu, XRush, JSKaraoglu, DKrasnewich, DLubinsky, MSWaechter, CJGilmore, RFreeze, HHHum Mutat 22:144-5022742743Pubmed2012Mutations in DPAGT1 cause a limb-girdle congenital myasthenic syndrome with tubular aggregatesBelaya, KatsiarynaFinlayson, SarahSlater, Clarke RCossins, JudithLiu, Wei WeiMaxwell, SusanMcGowan, SJMaslau, SiarheiTwigg, SRWalls, Timothy JPascual Pascual, Samuel IPalace, JacquelineBeeson, DavidAm. J. Hum. Genet. 91:193-201LEFT-TO-RIGHT2.4.1.141ALG13:ALG14 transfers GlcNAc from UDP-GlcNAc to GlcNAcDOLPAddition of the second N-acetyl-glucosamine to the N-glycan precursorThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>N,N'-DCDOLDPN,N'-diacetylchitobiosyldiphosphodolicholdiacetylchitobiosyldiphosphodolicholReactome DB_ID: 449293diacetylchitobiosyldiphosphodolichol [ChEBI:18341]diacetylchitobiosyldiphosphodolicholChEBICHEBI:18341Reactome Database ID Release 75449293Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449293ReactomeR-ALL-4492933Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449293.3ACTIVATIONALG13:ALG14Reactome DB_ID: 9965411E2RPL3ALG13(1-165)Reactome DB_ID: 9965405UniProt:E2RPL3UniProtE2RPL31EQUAL165EQUALReactome Database ID Release 759965405Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965405ReactomeR-CFA-4493291Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449329.11ALG14ALG14J9NWV0Reactome DB_ID: 9965409UniProt:J9NWV0ALG14UniProtJ9NWV01EQUAL216EQUALReactome Database ID Release 759965409Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965409ReactomeR-CFA-4492661Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449266.11Reactome Database ID Release 759965411Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965411ReactomeR-CFA-4493261Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449326.1GENE ONTOLOGYGO:0004577Reactome Database ID Release 759965412Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965412Reactome Database ID Release 759965414Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965414ReactomeR-CFA-4462071Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446207.1A second N-acetylglucosamine is added to the N-glycan precursor via a beta-1,4 linkage. This reaction is catalyzed by the ALG13:ALG14 complex, in which ALG13 functions as the catalyst and ALG14 functions as a membrane anchor which recruits ALG13 to the cytosolic face of ER (Gao XD et al, 2005).16100110Pubmed2005Alg14 recruits Alg13 to the cytoplasmic face of the endoplasmic reticulum to form a novel bipartite UDP-N-acetylglucosamine transferase required for the second step of N-linked glycosylationGao, XDTachikawa, HSato, TJigami, YDean, NJ Biol Chem 280:36254-62LEFT-TO-RIGHT2.4.1.142Addition of the first mannose to the N-glycan precursor by ALG1This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>MDCDDbeta-D-mannosyldiacetylchitobiosyldiphosphodolicholReactome DB_ID: 449283beta-D-mannosyldiacetylchitobiosyldiphosphodolichol [ChEBI:18396]beta-D-mannosyldiacetylchitobiosyldiphosphodolicholChEBICHEBI:18396Reactome Database ID Release 75449283Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449283ReactomeR-ALL-4492833Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449283.3ACTIVATIONALG1ALG1E2QXG7Reactome DB_ID: 9965441UniProt:E2QXG7ALG1UniProtE2QXG71EQUAL464EQUALReactome Database ID Release 759965441Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965441ReactomeR-CFA-4492231Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449223.1GENE ONTOLOGYGO:0004578Reactome Database ID Release 759965442Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965442Reactome Database ID Release 759965444Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965444ReactomeR-CFA-4462181Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446218.1A mannose is added to the N-glycan precursor via a beta-1,4 linkage. The reaction is catalyzed by ALG1 (Takahashi T et al, 2000). Defects in ALG1 lead to congenital disorder of glycosylation type 1K (CDG1K) (Schwarz et al. 2004; Kranz et al. 2004; Grubenmann et al. 2004).14973778Pubmed2004Deficiency of GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase causes congenital disorder of glycosylation type IkSchwarz, MThiel, CLübbehusen, JDorland, Bde Koning, Tvon Figura, KLehle, LKörner, CAm J Hum Genet 74:472-8114709599Pubmed2004Deficiency of the first mannosylation step in the N-glycosylation pathway causes congenital disorder of glycosylation type IkGrubenmann, CEFrank, CGHülsmeier, AJSchollen, EMatthijs, GMayatepek, EBerger, EGAebi, MHennet, THum Mol Genet 13:535-4210704531Pubmed2000Cloning of the human cDNA which can complement the defect of the yeast mannosyltransferase I-deficient mutant alg 1Takahashi, THonda, RNishikawa, YGlycobiology 10:321-714973782Pubmed2004Congenital disorder of glycosylation type Ik (CDG-Ik): a defect of mannosyltransferase IKranz, CDenecke, JLehle, LSohlbach, KJeske, SMeinhardt, FRossi, RGudowius, SMarquardt, TAm J Hum Genet 74:545-51LEFT-TO-RIGHT2.4.1Addition of a second mannose to the N-glycan precursor by ALG2This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>(GlcNAc)2 (Man)2 (PP-Dol)1alpha-D-Man-(1->6)-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-D-GlcNAc(PP-Dol)Reactome DB_ID: 449737alpha-D-Man-(1->6)-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-D-GlcNAc(PP-Dol) [ChEBI:59085]alpha-D-Man-(1->6)-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-D-GlcNAc(PP-Dol)ChEBICHEBI:59085Reactome Database ID Release 75449737Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449737ReactomeR-ALL-4497373Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449737.3ACTIVATIONALG2ALG2F6X6I6Reactome DB_ID: 9965418UniProt:F6X6I6ALG2UniProtF6X6I61EQUAL416EQUALReactome Database ID Release 759965418Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965418ReactomeR-CFA-4493361Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449336.1GENE ONTOLOGYGO:0000033Reactome Database ID Release 759965419Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965419Reactome Database ID Release 759965421Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965421ReactomeR-CFA-4462081Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446208.1A second mannose is added to the N-glycan precursor via an alpha-1,3 linkage. The reaction is catalyzed by the mannosyltransferase ALG2. This is a bifunctional enzyme with both alpha 1,3- and alpha 1,6-mannosyltransferase activities. In humans, only the alpha 1,3 activity used in this reaction has been elucidated (Thiel et al. 2003). Defects in ALG2 are the cause of ALG2-CDG (CDG-1i; MIM:607906) (Thiel et al. 2003).12684507Pubmed2003A new type of congenital disorders of glycosylation (CDG-Ii) provides new insights into the early steps of dolichol-linked oligosaccharide biosynthesisThiel, CSchwarz, MPeng, JGrzmil, MHasilik, MBraulke, TKohlschütter, Avon Figura, KLehle, LKörner, CJ Biol Chem 278:22498-505LEFT-TO-RIGHT2.4.1Addition of the sixth mannose to the N-glycan precursor by ALG3ALG3 transfers Man to N-glycan precursor (GlcNAc)2 (Man)5 (PP-Dol)1This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>DOLP-ManDolichyl phosphate D-mannosedolichyl D-mannosyl phosphateReactome DB_ID: 449229integral component of lumenal side of endoplasmic reticulum membraneGENE ONTOLOGYGO:0071556Reactome Database ID Release 75449229Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449229ReactomeR-ALL-4492293Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449229.3glycan G00006(GlcNAc)2 (Man)5 (PP-Dol)1Reactome DB_ID: 449298glycan G00006 [ChEBI:53022]glycan G00006ChEBICHEBI:53022Reactome Database ID Release 75449298Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449298ReactomeR-ALL-4492983Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449298.3glycan G10595(GlcNAc)2 (Man)6 (PP-Dol)1Reactome DB_ID: 449315glycan G10595 [ChEBI:53023]glycan G10595ChEBICHEBI:53023Reactome Database ID Release 75449315Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449315ReactomeR-ALL-4493153Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449315.3DOLPDolichol phosphatedolichyl phosphateReactome DB_ID: 449311Reactome Database ID Release 75449311Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449311ReactomeR-ALL-4493113Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449311.3ACTIVATIONALG3ALG3E2RQY8Reactome DB_ID: 9965336UniProt:E2RQY8ALG3UniProtE2RQY81EQUAL438EQUALReactome Database ID Release 759965336Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965336ReactomeR-CFA-4492871Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449287.1Reactome Database ID Release 759965337Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965337Reactome Database ID Release 759965343Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965343ReactomeR-CFA-4461881Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446188.1The sixth mannose is added to the N-glycan precursor. This reaction occurs in the ER lumen and uses a different mannose donor (dolichyl-phosphate-mannose) than the previous steps. It has been proposed that ALG3, along with all the mannosyl- and glucosyltransferases in the N-glycan biosynthesis pathway that use dolichyl-phosphate-mannose or dolichyl-phosphate-glucose as donor, derive from duplications of a common ancestral enzyme (Oriol et al. 2002). Defects in ALG3 are associated with Congenital Disorder of Glycosylation 1D (CDG1D) (Sun et al. 2005).15840742Pubmed2005Congenital disorder of glycosylation id presenting with hyperinsulinemic hypoglycemia and islet cell hyperplasiaSun, LEklund, EAChung, WKWang, CCohen, JFreeze, HHJ Clin Endocrinol Metab 90:4371-512200473Pubmed2002Common origin and evolution of glycosyltransferases using Dol-P-monosaccharides as donor substrateOriol, RMartinez-Duncker, IChantret, IMollicone, RCodogno, PMol Biol Evol 19:1451-63ACTIVATIONReactome Database ID Release 759965344Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965344MPDU1MPDU1E2R6F8Reactome DB_ID: 9965341UniProt:E2R6F8MPDU1UniProtE2R6F82EQUAL247EQUALReactome Database ID Release 759965341Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965341ReactomeR-CFA-46284281Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-4628428.1LEFT-TO-RIGHT2.4.1Addition of the seventh mannose to the N-glycan precursor by ALG9ALG9 transfers Man to N-glycan precursor (GlcNAc)2 (Man)6 (PP-Dol)1This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>(GlcNAc)2 (Man)7 (PP-Dol)1alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol)Reactome DB_ID: 449312alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol) [ChEBI:59088]alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol)ChEBICHEBI:59088Reactome Database ID Release 75449312Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449312ReactomeR-ALL-4493123Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449312.3ACTIVATIONALG9FDXACB1J9PAV7Reactome DB_ID: 9965432UniProt:J9PAV7FDXACB1UniProtJ9PAV71EQUAL611EQUALReactome Database ID Release 759965432Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965432ReactomeR-CFA-4492411Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449241.1GENE ONTOLOGYGO:0000026Reactome Database ID Release 759965433Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965433Reactome Database ID Release 759965435Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965435ReactomeR-CFA-4462151Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446215.1The seventh mannose is added to the N-glycan precursor. This reaction occurs in the ER lumen and uses dolichyl phosphate D-mannose as the mannose donor with ALG9 mediating the reaction. Defects in ALG9 are the cause of congenital disorder of glycosylation type 1L (CDG1L) (Frank CG et al, 2004; Weinstein M et al, 2005). For many years ALG9 has been thought to be involved in bipolar affective disorder (Baysal BE et al, 2002), but this hypothesis has been proven wrong (Baysal BE et al, 2006).16859551Pubmed2006Common variations in ALG9 are not associated with bipolar I disorder: a family-based studyBaysal, BEWillett-Brozick, JEBacanu, SADetera-Wadleigh, SNimgaonkar, VLBehav Brain Funct 2:2515945070Pubmed2005CDG-IL: an infant with a novel mutation in the ALG9 gene and additional phenotypic featuresWeinstein, MSchollen, EMatthijs, GNeupert, CHennet, TGrubenmann, CEFrank, CGAebi, MClarke, JTGriffiths, ASeargeant, LPoplawski, NAm J Med Genet A 136:194-712030331Pubmed2002A mannosyltransferase gene at 11q23 is disrupted by a translocation breakpoint that co-segregates with bipolar affective disorder in a small familyBaysal, BEWillett-Brozick, JEBadner, JACorona, WFerrell, RENimgaonkar, VLDetera-Wadleigh, SDNeurogenetics 4:43-5315148656Pubmed2004Identification and functional analysis of a defect in the human ALG9 gene: definition of congenital disorder of glycosylation type ILFrank, CGGrubenmann, CEEyaid, WBerger, EGAebi, MHennet, TAm J Hum Genet 75:146-50ACTIVATIONLEFT-TO-RIGHT2.4.1Addition of the eighth mannose to the N-glycan precursor by ALG12ALG12 transfers Man to N-glycan precursor (GlcNAc)2 (Man)7 (PP-Dol)1This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>(GlcNAc)2 (Man)8 (PP-Dol)1alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->6)-[alpha-Man-(1->2)-alpha-Man-(1->3)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol)Reactome DB_ID: 449365alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->6)-[alpha-Man-(1->2)-alpha-Man-(1->3)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol) [ChEBI:59091]alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->6)-[alpha-Man-(1->2)-alpha-Man-(1->3)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol)ChEBICHEBI:59091Reactome Database ID Release 75449365Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449365ReactomeR-ALL-4493653Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449365.3ACTIVATIONALG12ALG12E2R1H6Reactome DB_ID: 9965362UniProt:E2R1H6ALG12UniProtE2R1H61EQUAL488EQUALReactome Database ID Release 759965362Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965362ReactomeR-CFA-4493481Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449348.1GENE ONTOLOGYGO:0000030Reactome Database ID Release 759965363Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965363Reactome Database ID Release 759965365Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965365ReactomeR-CFA-4461981Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446198.1The eighth mannose is added to the N-glycan precursor. This reaction occurs in the ER lumen and uses dolichyl phosphate D-mannose as a mannose donor. Defects in ALG12 are the cause of congenital disorder of glycosylation type 1G (CDG1G) (Chantret I et al, 2002).11983712Pubmed2002Congenital disorders of glycosylation type Ig is defined by a deficiency in dolichyl-P-mannose:Man7GlcNAc2-PP-dolichyl mannosyltransferaseChantret, IDupré, TDelenda, CBucher, SDancourt, JBarnier, ACharollais, AHeron, DBader-Meunier, BDanos, OSeta, NDurand, GOriol, RCodogno, PMoore, SEJ Biol Chem 277:25815-22ACTIVATIONLEFT-TO-RIGHT2.4.1Addition of the last mannose to the N-glycan precursor by ALG9ALG9 transfers Man to N-glycan precursor (GlcNAc)2 (Man)8 (PP-Dol)1This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>(GlcNAc)2 (Man)9 (PP-Dol)1alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol)Reactome DB_ID: 449371alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol) [ChEBI:37637]alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol)ChEBICHEBI:37637Reactome Database ID Release 75449371Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449371ReactomeR-ALL-4493713Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449371.3ACTIVATIONReactome Database ID Release 759965437Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965437ReactomeR-CFA-4462161Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446216.1The last mannose is added to the N-glycan precursor. This reaction occurs in the ER lumen, uses Dolichyl phosphate D-mannose as the mannose donor, and is catalyzed by ALG9. Defects in ALG9 are the cause of congenital disorder of glycosylation type 1L (CDG1L) (Frank CG et al, 2004; Weinstein M et al, 2005). For many years ALG9 was thought to be involved in bipolar affective disorder (Baysal BE et al, 2002), but this hypothesis has been proven wrong (Baysal BE et al, 2006).ACTIVATIONLEFT-TO-RIGHT2.4.1Addition of the first glucose to the N-glycan precursor by ALG6This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>DbGPdolichyl beta-D-glucosyl phosphateReactome DB_ID: 532542Reactome Database ID Release 75532542Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=532542ReactomeR-ALL-5325423Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-532542.3(Glc)1 (GlcNAc)2 (Man)9 (PP-Dol)1alpha-Gal-(1->3)-alpha-Glc-(1->3)-alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol)Reactome DB_ID: 449649alpha-Gal-(1->3)-alpha-Glc-(1->3)-alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol) [ChEBI:59081]alpha-Gal-(1->3)-alpha-Glc-(1->3)-alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol)ChEBICHEBI:59081Reactome Database ID Release 75449649Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449649ReactomeR-ALL-4496493Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449649.3ACTIVATIONALG6ALG6E2R652Reactome DB_ID: 9965389UniProt:E2R652ALG6UniProtE2R6521EQUAL507EQUALReactome Database ID Release 759965389Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965389ReactomeR-CFA-4496451Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449645.1GENE ONTOLOGYGO:0004583Reactome Database ID Release 759965390Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965390Reactome Database ID Release 759965392Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965392ReactomeR-CFA-4462021Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446202.1The first glucose is added to the N-glycan precursor, mediated by ALG6. Defects in ALG6 are associated with CDG-Ic disorder (Imbach T et al, 1999; Sun L et al, 2005). The donor is a dolichol-phosphate-glucose (synthesized by ALG5). 16007612Pubmed2005Clinical and molecular characterization of the first adult congenital disorder of glycosylation (CDG) type Ic patientSun, LEklund, EAVan Hove, JLFreeze, HHThomas, JAAm J Med Genet A 137:22-6LEFT-TO-RIGHT2.4.1Addition of a second glucose to the N-glycan precursor by ALG8This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>glycan G10599(Glc)2 (GlcNAc)2 (Man)9 (PP-Dol)1Reactome DB_ID: 449638glycan G10599 [ChEBI:53020]glycan G10599ChEBICHEBI:53020Reactome Database ID Release 75449638Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=449638ReactomeR-ALL-4496383Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-449638.3ACTIVATIONALG8ALG8E2RM31Reactome DB_ID: 9965348UniProt:E2RM31ALG8UniProtE2RM311EQUAL526EQUALReactome Database ID Release 759965348Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965348ReactomeR-CFA-4496401Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-449640.1Reactome Database ID Release 759965349Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965349Reactome Database ID Release 759965351Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9965351ReactomeR-CFA-4461891Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446189.1The second glucose (supplied from the donor dolichol-phosphate-glucose) is added to the N-glycan precursor, mediated by ALG8 (Schollen E et al, 2004). Defects in ALG8 are the cause of congenital disorder of glycosylation type 1H (CDG1H) (Schollen E et al, 2004; Sun L et al, 2005).15235028Pubmed2004Clinical and molecular features of three patients with congenital disorders of glycosylation type Ih (CDG-Ih) (ALG8 deficiency)Schollen, EFrank, CGKeldermans, LReyntjens, RGrubenmann, CEClayton, Peter TWinchester, BGSmeitink, JWevers, RAAebi, MHennet, TMatthijs, GJ Med Genet 41:550-6Reactome Database ID Release 7510027405Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10027405ReactomeR-CFA-4461931Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-446193.1GENE ONTOLOGYGO:0006488N-linked glycosylation commences with the 14-step synthesis of a dolichol lipid-linked oligosaccharide (LLO) consisting of 14 sugars (2 core GlcNAcs, 9 mannoses and 3 terminal GlcNAcs). This pathway is highly conserved in eukaryotes, and a closely related pathway is found in many eubacteria and Archaea. Mutations in the genes associated with N-glycan precursor synthesis lead to a diverse group of disorders collectively known as Congenital Disorders of Glycosylation (type I and II) (Sparks et al. 1993). The phenotypes of these disorders reflect the important role that N-glycosylation has during development, controlling the folding and the properties of proteins in the secretory pathway, and proteins that mediate cell-to-cell interactions or timing of development.1993Congenital Disorders of Glycosylation OverviewSparks, SEKrasnewich, DMPagon, RABird, TCDolan, CRStephens, KCongenital Disorders of Glycosylation Overview (Book): N-glycan trimming in the ER and Calnexin/Calreticulin cycleThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Calnexin/calreticulin cycleThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>ER Quality Control Compartment (ERQC)This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>LEFT-TO-RIGHTUb-unfolded protein:(GlcNAc)2 (Man)9-5 translocates from ERQC to cytosolGlycoproteins with major folding defects are sent to degradation by OS9:SEL1:SYVN1 dimer:DERL2This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Ub-unfolded protein:(GlcNAc)2 (Man)9-5Reactome DB_ID: 9973678endoplasmic reticulum quality control compartmentGENE ONTOLOGYGO:0044322Ghost homologue of unfolded protein:(GlcNAc)2 (Man)9-5Reactome DB_ID: 9973676Reactome Database ID Release 759973676Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9973676ReactomeR-CFA-88528451Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8852845.11Converted from EntitySet in ReactomeUbubiquitinReactome DB_ID: 9933738RPS27ARPS27A(1-76)F1PEZ4Reactome DB_ID: 9933704UniProt:F1PEZ4RPS27AUniProtF1PEZ41EQUAL76EQUALReactome Database ID Release 759933704Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9933704ReactomeR-CFA-9392051Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-939205.1UBA52UBA52(1-76)P63050Reactome DB_ID: 9933708UniProt:P63050UBA52UniProtP630501EQUAL76EQUALReactome Database ID Release 759933708Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9933708ReactomeR-CFA-9392031Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-939203.1J9NVT1UBB(1-76)Reactome DB_ID: 9933712UniProt:J9NVT1UniProtJ9NVT11EQUAL76EQUALReactome Database ID Release 759933712Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9933712ReactomeR-CFA-9392141Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-939214.1J9NVT1UBB(77-152)Reactome DB_ID: 993371477EQUAL152EQUALReactome Database ID Release 759933714Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9933714ReactomeR-CFA-9392131Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-939213.1J9NVT1UBB(153-228)Reactome DB_ID: 9933716153EQUAL228EQUALReactome Database ID Release 759933716Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9933716ReactomeR-CFA-9392301Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-939230.1UBBUBC(1-76)F1PDG4Reactome DB_ID: 9933720UniProt:F1PDG4UBBUniProtF1PDG41EQUAL76EQUALReactome Database ID Release 759933720Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9933720ReactomeR-CFA-9391881Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-939188.1Reactome Database ID Release 759933738Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9933738ReactomeR-CFA-1135951Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-113595.11Reactome Database ID Release 759973678Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9973678ReactomeR-CFA-88672871Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8867287.1Ub-unfolded protein:(GlcNAc)2 (Man)9-5Reactome DB_ID: 9973680Converted from EntitySet in Reactomeunfolded protein:(GlcNAc)2 (Man)9-5Reactome DB_ID: 8852851unfolded protein:(GlcNAc)2 (Man)9Reactome DB_ID: 8852849(GlcNAc)2 (Man)9N-{alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)-[alpha-Man-(1->6)-[alpha-Man-(1->2)-alpha-Man-(1->2)]-alpha-Man-(1->3)]-beta-Man-(1->4)-beta-GlcnAc-(1->4)-beta-GlcNAc}-L-Asn residueReactome DB_ID: 8852846N-{alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)-[alpha-Man-(1->6)-[alpha-Man-(1->2)-alpha-Man-(1->2)]-alpha-Man-(1->3)]-beta-Man-(1->4)-beta-GlcnAc-(1->4)-beta-GlcNAc}-L-Asn residue [ChEBI:59092]N-{alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)-[alpha-Man-(1->6)-[alpha-Man-(1->2)-alpha-Man-(1->2)]-alpha-Man-(1->3)]-beta-Man-(1->4)-beta-GlcnAc-(1->4)-beta-GlcNAc}-L-Asn residueChEBICHEBI:59092Reactome Database ID Release 758852846Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8852846ReactomeR-ALL-88528463Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8852846.31unfolded proteinReactome DB_ID: 1022114Reactome Database ID Release 751022114Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1022114ReactomeR-ALL-10221142Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-1022114.2ChEBI360801Reactome Database ID Release 758852849Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8852849ReactomeR-ALL-88528492Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8852849.2unfolded protein:(GlcNAc)2 (Man)5Reactome DB_ID: 6782684(GlcNAc)2 (Man)5N-{alpha-Man-(1->3)-[alpha-Man-(1->3)-[alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-beta-GlcNac}-L-Asn residueReactome DB_ID: 6782686N-{alpha-Man-(1->3)-[alpha-Man-(1->3)-[alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-beta-GlcNac}-L-Asn residue [ChEBI:59087]N-{alpha-Man-(1->3)-[alpha-Man-(1->3)-[alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-beta-GlcNac}-L-Asn residueChEBICHEBI:59087Reactome Database ID Release 756782686Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6782686ReactomeR-ALL-67826863Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6782686.311Reactome Database ID Release 756782684Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6782684ReactomeR-ALL-67826842Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6782684.2unfolded protein:(GlcNAc)2 (Man)8aReactome DB_ID: 8852854(GlcNAc)2 (Man)8aN(4)-{alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlaNAc-(1->4)-beta-D-GlcNAc}-Asn residueN(4)-{alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-beta-D-GlcNAc}-Asn residueReactome DB_ID: 1022112N(4)-{alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-beta-D-GlcNAc}-Asn residue [ChEBI:60627]N(4)-{alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-beta-D-GlcNAc}-Asn residueChEBICHEBI:60627Reactome Database ID Release 751022112Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1022112ReactomeR-ALL-10221123Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-1022112.311Reactome Database ID Release 758852854Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8852854ReactomeR-ALL-88528542Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8852854.2unfolded protein:(GlcNAc)2 (Man)8bReactome DB_ID: 88528531(GlcNAc)2 (Man)8bN(4)-{alpha-D-Manp-(1->2)-alpha-D-Manp-(1->2)-alpha-D-Manp-(1->3)-[alpha-D-Manp-(1->3)-[alpha-D-Manp-(1->2)-alpha-D-Manp-(1->6)]-alpha-D-Manp-(1->6)]-beta-D-Manp-(1->4)-beta-D-GlcpNAc-(1->4)-beta-D-GlcpNAc}-Asn residueReactome DB_ID: 8852850N(4)-{alpha-D-Manp-(1->2)-alpha-D-Manp-(1->2)-alpha-D-Manp-(1->3)-[alpha-D-Manp-(1->3)-[alpha-D-Manp-(1->2)-alpha-D-Manp-(1->6)]-alpha-D-Manp-(1->6)]-beta-D-Manp-(1->4)-beta-D-GlcpNAc-(1->4)-beta-D-GlcpNAc}-Asn residue [ChEBI:60628]N(4)-{alpha-D-Manp-(1->2)-alpha-D-Manp-(1->2)-alpha-D-Manp-(1->3)-[alpha-D-Manp-(1->3)-[alpha-D-Manp-(1->2)-alpha-D-Manp-(1->6)]-alpha-D-Manp-(1->6)]-beta-D-Manp-(1->4)-beta-D-GlcpNAc-(1->4)-beta-D-GlcpNAc}-Asn residueChEBICHEBI:60628Reactome Database ID Release 758852850Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8852850ReactomeR-ALL-88528503Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8852850.31Reactome Database ID Release 758852853Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8852853ReactomeR-ALL-88528532Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8852853.2unfolded protein:(GlcNAc)2 (Man)8cReactome DB_ID: 8852847(GlcNAc)2 (Man)8cN(4)-{alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-beta-D-GlcNAc}-Asn residueReactome DB_ID: 8852848N(4)-{alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-beta-D-GlcNAc}-Asn residue [ChEBI:60629]N(4)-{alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc-(1->4)-beta-D-GlcNAc}-Asn residueChEBICHEBI:60629Reactome Database ID Release 758852848Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8852848ReactomeR-ALL-88528483Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8852848.311Reactome Database ID Release 758852847Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8852847ReactomeR-ALL-88528472Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8852847.2Reactome Database ID Release 758852851Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8852851ReactomeR-ALL-88528512Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8852851.211Reactome Database ID Release 759973680Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9973680ReactomeR-CFA-88668941Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8866894.1Reactome Database ID Release 759973682Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9973682ReactomeR-CFA-10221271Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-1022127.1Proteins with major folding defects are extracted from futile folding cycles in the calnexin chaperone system and the ER Quality Control Compartment, and are translocated back to the cytosol for ER-associated degradation (ERAD). The N-glycan is used as a signal to distinguish proteins to be degraded, by direct binding to a ubiquitin ligase complex composed of, minimally, an E3 ubiquitin-protein ligase, protein sel-1 homolog 1 (SEL1L), derlin-2 (DERL2) and protein OS-9 (OS9) (Christianson et al. 2008, Bernasconi et al. 2008, Alcock & Swanton 2009; review Olzmann et al. 2013). The mechanism of translocation is unknown.18264092Pubmed2008OS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERADChristianson, John CShaler, Thomas ATyler, Ryan EKopito, Ron RNat. Cell Biol. 10:272-8218417469Pubmed2008A dual task for the Xbp1-responsive OS-9 variants in the mammalian endoplasmic reticulum: inhibiting secretion of misfolded protein conformers and enhancing their disposalBernasconi, RiccardoPertel, ThomasLuban, JeremyMolinari, MaurizioJ. Biol. Chem. 283:16446-5419084021Pubmed2009Mammalian OS-9 is upregulated in response to endoplasmic reticulum stress and facilitates ubiquitination of misfolded glycoproteinsAlcock, FelicitySwanton, EileithyiaJ. Mol. Biol. 385:1032-4223232094Pubmed2013The mammalian endoplasmic reticulum-associated degradation systemOlzmann, James AKopito, Ron RChristianson, John CCold Spring Harb Perspect Biol 5:Reactome Database ID Release 7510028697Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10028697ReactomeR-CFA-9010321Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-901032.1GENE ONTOLOGYGO:1904380Proteins that are released from the CNX or CRT complex with folding defects accumulate in a compartment of the ER called ERQC (Kamhi-Nesher et al. 2001). Here, the enzymes UGGG1 or UGGG2 are able to recognize glycoproteins with minor folding process and re-add the glucose on the alpha,1,3 branch; this is a signal for the transport of these glycoproteins back to the ER, where they can interact again with CNX or CRT in order to achieve a correct folding. At the same time that the glycoprotein is in the ERQC, the enzyme ER mannosidase I progressively removes the mannoses at positions 1A, 2A, B, C on N-glycans; when the mannose on 1A is trimmed, UDP-Glc:glycoprotein glucosyltransferases 1 and 2 (UGGT1 and 2) are no longer able to re-add the glucose, and therefore the protein is destined for ERAD. Glycoproteins subject to endoplasmic reticulum-associated degradation (ERAD) undergo reglucosylation, deglucosylation, and mannose trimming to yield Man6GlcNAc2 and Man5GlcNAc2. These structures lack the mannose residue that is the acceptor of glucose transferred by UGGT1 and 2. For years it has been thought that the removal of the mannose in position B of the N-glycan was the signal to direct proteins to degradation. However, this mechanism has been described better by Avezov et al (Avezov et al. 2008) and it has been demonstrated that even glycoproteins with Man8 or Man7 glycans can be re-glucosylated and interact again with CNX or CRT (for a review on this topic, see Lederkremer 2009 and Maattanen P et al, 2010).20347046Pubmed2010Protein quality control in the ER: the recognition of misfolded proteinsMäättänen, PGehring, KBergeron, JJThomas, DYSemin Cell Dev Biol 21:500-1119616933Pubmed2009Glycoprotein folding, quality control and ER-associated degradationLederkremer, GZCurr Opin Struct Biol 19:515-2311408579Pubmed2001A novel quality control compartment derived from the endoplasmic reticulumKamhi-Nesher, SShenkman, MTolchinsky, SFromm, SVEhrlich, RLederkremer, GZMol Biol Cell 12:1711-2318003979Pubmed2008Endoplasmic reticulum (ER) mannosidase I is compartmentalized and required for N-glycan trimming to Man5-6GlcNAc2 in glycoprotein ER-associated degradationAvezov, EFrenkel, ZEhrlich, MHerscovics, ALederkremer, GZMol Biol Cell 19:216-25Reactome Database ID Release 7510028699Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10028699ReactomeR-CFA-9010421Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-901042.1The unfolded protein is recognized by a chaperon protein (calnexin or calreticulin) and the folding process starts. The binding of these protein requires a mono-glucosylated glycan (Caramelo JJ and Parodi AJ, 2008), but in certain cases can occur even in the absence of glycosylation (Ireland BS et al, 2008).18303019Pubmed2008Getting in and out from calnexin/calreticulin cyclesCaramelo, JJParodi, AJJ Biol Chem 283:10221-518337472Pubmed2008Lectin-deficient calreticulin retains full functionality as a chaperone for class I histocompatibility moleculesIreland, BSBrockmeier, UHowe, CMElliott, TWilliams, DBMol Biol Cell 19:2413-23LEFT-TO-RIGHT3.5.1.52Deglycosylation complex hydrolyses N-glycans from unfolded glycoproteinsThis event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Converted from EntitySet in Reactome(GlcNAc)2 (Man)9-5Reactome DB_ID: 8852852Reactome Database ID Release 758852852Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8852852ReactomeR-ALL-88528524Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8852852.4ACTIVATIONactiveUnit: #Protein84Deglycosylation complexReactome DB_ID: 10015072PSMC1PSMC1F1PQ40Reactome DB_ID: 9933816UniProt:F1PQ40PSMC1UniProtF1PQ402EQUAL440EQUALReactome Database ID Release 759933816Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9933816ReactomeR-CFA-687681Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-68768.11RAD23BRAD23BE2R4A5Reactome DB_ID: 10015070UniProt:E2R4A5RAD23BUniProtE2R4A51EQUAL409EQUALReactome Database ID Release 7510015070Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015070ReactomeR-CFA-88505891Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8850589.11UBXN1UBXN1J9NVT9Reactome DB_ID: 10015068UniProt:J9NVT9UBXN1UniProtJ9NVT92EQUAL297EQUALReactome Database ID Release 7510015068Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015068ReactomeR-CFA-88505881Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8850588.11VCP hexamerReactome DB_ID: 9996640VCPVCPE2RLQ9Reactome DB_ID: 9996081UniProt:E2RLQ9VCPUniProtE2RLQ92EQUAL806EQUALReactome Database ID Release 759996081Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9996081ReactomeR-CFA-11812511Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-1181251.16Reactome Database ID Release 759996640Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=9996640ReactomeR-CFA-53623641Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-5362364.11AMFRAMFRF1PK72Reactome DB_ID: 10015056UniProt:F1PK72AMFRUniProtF1PK721EQUAL643EQUALReactome Database ID Release 7510015056Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015056ReactomeR-CFA-9759991Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-975999.11NGLY1NGLY1E2R815Reactome DB_ID: 10015064UniProt:E2R815NGLY1UniProtE2R8152EQUAL654EQUALReactome Database ID Release 7510015064Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015064ReactomeR-CFA-88505911Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8850591.11DERL1DERL1E2RT23Reactome DB_ID: 10015060UniProt:E2RT23DERL1UniProtE2RT232EQUAL251EQUALReactome Database ID Release 7510015060Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015060ReactomeR-CFA-67818981Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-6781898.11Reactome Database ID Release 7510015072Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015072ReactomeR-CFA-88505901Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8850590.1GENE ONTOLOGYGO:0000224Reactome Database ID Release 7510015073Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015073Reactome Database ID Release 7510015075Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015075ReactomeR-CFA-88505941Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8850594.1Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase (NGLY1, aka PNGase) is a cytosolic peptide:N-glycanase which acts on N-glycoproteins generating free, unconjugated N-glycans and deglycosylated peptides in which the N-glycosylated asparagine residues are converted to aspartates. It is involved in the quality control system for misfolded glycoproteins exported to the cytosol that need to be targeted for degradation (Suzuki et al. 2016). NGLY1 is part of a complex that couples retrotranslocation, ubiquitination and deglycosylation. It is probably composed of NGLY1, UBX domain-containing protein 1 (UBXN1 aka SAKS1), E3 ubiquitin-protein ligase (AMFR), transitional endoplasmic reticulum ATPase (VCP), derlin-1 (DERL1), 26S protease regulatory subunit 4 (PSMC1) and UV excision repair protein RAD23 homolog B (RAD23B). NGLY1 interacts with the proteasome components RAD23B and PSMC1, directly with VCP and with DERL1, bringing it close to the endoplasmic reticulum membrane (Katiyar & Lennarz 2004, Song et al. 2005, McNeill et al. 2004, Ye et al. 2004, Katiyar et al. 2005).16168377Pubmed2005Gp78, a membrane-anchored ubiquitin ligase, associates with Insig-1 and couples sterol-regulated ubiquitination to degradation of HMG CoA reductaseSong, BLSever, NavdarDeBose-Boyd, RAMol. Cell 19:829-4015362974Pubmed2004A novel UBA and UBX domain protein that binds polyubiquitin and VCP and is a substrate for SAPKsMcNeill, HKnebel, AxelArthur, J Simon CCuenda, AnaCohen, PBiochem. J. 384:391-40015358861Pubmed2004A complex between peptide:N-glycanase and two proteasome-linked proteins suggests a mechanism for the degradation of misfolded glycoproteinsKatiyar, SamikshaLi, GuangtaoLennarz, William JProc. Natl. Acad. Sci. U.S.A. 101:13774-915215856Pubmed2004A membrane protein complex mediates retro-translocation from the ER lumen into the cytosolYe, YihongShibata, YokoYun, ChiRon, DavidRapoport, Tom ANature 429:841-716055502Pubmed2005The retrotranslocation protein Derlin-1 binds peptide:N-glycanase to the endoplasmic reticulumKatiyar, SamikshaJoshi, ShivanjaliLennarz, William JMol. Biol. Cell 16:4584-9426611529Pubmed2016The cytoplasmic peptide:N-glycanase (NGLY1) - Structure, expression and cellular functionsSuzuki, TadashiHuang, ChengchengFujihira, HaruhikoGene 577:1-7LEFT-TO-RIGHT3.2.1.96ENGASE hydrolyses unfolded protein:(GlcNAc)2 (Man(9-5)This event has been computationally inferred from an event that has been demonstrated in another species.<p>The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.<p><a href='/electronic_inference_compara.html' target = 'NEW'>More details and caveats of the event inference in Reactome.</a> For details on PANTHER see also: <a href='http://www.pantherdb.org/about.jsp' target='NEW'>http://www.pantherdb.org/about.jsp</a>Converted from EntitySet in ReactomeGlcNAc (Man)9-5Reactome DB_ID: 8853381GlcNAc (Man)5alpha-D-Man-(1->3)-[alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcReactome DB_ID: 6799543alpha-D-Man-(1->3)-[alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc [ChEBI:91280]alpha-D-Man-(1->3)-[alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcChEBICHEBI:91280Reactome Database ID Release 756799543Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6799543ReactomeR-ALL-67995433Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6799543.3GlcNAc (Man)8aalpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcReactome DB_ID: 8853369alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc [ChEBI:91281]alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcChEBICHEBI:91281Reactome Database ID Release 758853369Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8853369ReactomeR-ALL-88533693Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8853369.3GlcNAc (Man)8balpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcReactome DB_ID: 8853374alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc [ChEBI:91282]alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcChEBICHEBI:91282Reactome Database ID Release 758853374Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8853374ReactomeR-ALL-88533743Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8853374.3GlcNAc (Man)8calpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcReactome DB_ID: 8853378alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc [ChEBI:91283]alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->6)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcChEBICHEBI:91283Reactome Database ID Release 758853378Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8853378ReactomeR-ALL-88533783Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8853378.3GlcNAc (Man)9alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcReactome DB_ID: 6799540alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAc [ChEBI:91284]alpha-D-Man-(1->2)-alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->3)-[alpha-D-Man-(1->2)-alpha-D-Man-(1->6)]-alpha-D-Man-(1->6)]-beta-D-Man-(1->4)-beta-D-GlcNAcChEBICHEBI:91284Reactome Database ID Release 756799540Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6799540ReactomeR-ALL-67995403Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-6799540.3Reactome Database ID Release 758853381Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8853381ReactomeR-ALL-88533812Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8853381.2unfolded protein:GlcNAcReactome DB_ID: 8853371Reactome Database ID Release 758853371Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=8853371ReactomeR-ALL-88533713Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-8853371.3ChEBI33837ACTIVATIONENGASEENGASEF1PJY1Reactome DB_ID: 10015655UniProt:F1PJY1ENGASEUniProtF1PJY11EQUAL743EQUALReactome Database ID Release 7510015655Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015655ReactomeR-CFA-88533861Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8853386.1GENE ONTOLOGYGO:0033925Reactome Database ID Release 7510015656Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015656Reactome Database ID Release 7510015658Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10015658ReactomeR-CFA-88533791Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-8853379.1Two cytosolic deglycosylating systems can generate free oligosaccharides from glycoproteins; a deglycosylation complex that hydrolyses the whole glycan from an unfolded protein and cytosolic endo-beta-N-acetylglucosaminidase (ENGASE) that catalyses the endohydrolysis of -(Man(GlcNAc)2)Asn- structures in glycoproteins, leaving one N-acetyl-D-glucosamine (GlcNAc) residue attached to the protein (Suzuki et al. 2002). Glycans with a single GlcNAc at their reducing termini need to be further degraded by a cytosolic alpha-mannosidase, MAN2C1.12114544Pubmed2002Endo-beta-N-acetylglucosaminidase, an enzyme involved in processing of free oligosaccharides in the cytosolSuzuki, TadashiYano, KeiichiSugimoto, SeijiKitajima, KenLennarz, William JInoue, SadakoInoue, YasuoEmori, YasufumiProc. Natl. Acad. Sci. U.S.A. 99:9691-6Reactome Database ID Release 7510028701Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10028701ReactomeR-CFA-5326681Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-CFA-532668.1GENE ONTOLOGYGO:0006457After being synthesized in the ER membrane the 14-sugars lipid-linked oligosaccharide is co-translationally transferred to an unfolded protein, as described in the previous steps. After this point the N-glycan is progressively trimmed of the three glucoses and some of the mannoses before the protein is transported to the cis-Golgi. The role of these trimming reactions is that the N-glycan attached to an unfolded glycoprotein in the ER assume the role of 'tags' that direct the interactions of the glycoprotein with different elements that mediate its folding. The removal of the two outer glucoses leads to an N-glycan with only one glucose, which is a signal for the binding of either one of two chaperone proteins, calnexin (CNX) and calreticulin (CRT). These chaperones provide an environment where the protein can fold more easily. The interaction with these proteins is not transient and is terminated by the trimming of the last remaining glucose, after which the glycoprotein is released from CNX or CRT and directed to the ER Quality Control compartment (ERQC) if it still has folding defects, or transported to the Golgi if the folding is correct. The involvement of N-glycans in the folding quality control of proteins in the ER explains why this form of glycosylation is so important, and why defects in the enzymes involved in these reactions are frequently associated with congenital diseases. However, there are many unknown points in this process, as it is known that even proteins without N-glycosylation sites can be folded properly (Caramelo JJ and Parodi AJ, 2008).Transport to the Golgi and subsequent modificationThis event has been computat