BioPAX pathway converted from "Metabolism of carbohydrates" in the Reactome database. Carbohydrate metabolism Metabolism of carbohydrates 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> Glycogen metabolism 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> Glycogen synthesis 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-RIGHT 2.7.7.9 UTP + D-glucose 1-phosphate <=> pyrophosphate + UDP-glucose 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> G1P D-glucopyranose 1-phosphate(2-) D-Glucose 1-phosphate D-glucopyranose 1-phosphate alpha-D-Glucose 1-phosphate Cori ester D-Glucose alpha-1-phosphate Reactome DB_ID: 29548 cytosol GENE ONTOLOGY GO:0005829 D-glucopyranose 1-phosphate(2-) [ChEBI:57629] D-glucopyranose 1-phosphate(2-) 1-O-phosphonato-D-glucopyranose D-glucopyranose 1-phosphate anion D-glucopyranose 1-phosphate D-glucopyranose 1-phosphate dianion ChEBI CHEBI:57629 Reactome Database ID Release 81 29548 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29548 Reactome R-ALL-29548 4 Reactome stable identifier. Use this 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-29548.4 Reactome http://www.reactome.org UTP uridine 5'-triphosphate Uridine triphosphate Reactome DB_ID: 29494 UTP(4-) [ChEBI:46398] UTP(4-) UTP utp uridine 5'-triphosphate(4-) URIDINE 5'-TRIPHOSPHATE ChEBI CHEBI:46398 Reactome Database ID Release 81 29494 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29494 Reactome R-ALL-29494 3 Reactome stable identifier. Use this 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-29494.3 COMPOUND C00075 additional information MI MI:0361 PPi pyrophosphate diphosphoric acid pyrophosphoric acid diphosphate Reactome DB_ID: 111294 diphosphate(3-) [ChEBI:33019] diphosphate(3-) ChEBI CHEBI:33019 Reactome Database ID Release 81 111294 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=111294 Reactome R-ALL-111294 4 Reactome stable identifier. Use this 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-111294.4 COMPOUND C00013 UDP-Glc UDP-D-glucose(2-) UDP-glucose UDP-D-glucose UDPglucose Reactome DB_ID: 29410 UDP-D-glucose(2-) [ChEBI:58367] UDP-D-glucose(2-) UDP-D-glucose dianion UDP-Glc(2-) ChEBI CHEBI:58367 Reactome Database ID Release 81 29410 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29410 Reactome R-ALL-29410 4 Reactome stable identifier. Use this 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-29410.4 COMPOUND C00029 ACTIVATION 8xUGP2 Reactome DB_ID: 10207335 UGP2 UGP2 A0A286ZI08 Reactome DB_ID: 10207333 UniProt:A0A286ZI08 UGP2 Sus scrofa NCBI Taxonomy 9823 UniProt A0A286ZI08 1 EQUAL 508 EQUAL Reactome Database ID Release 81 10207333 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207333 Reactome R-SSC-453128 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453128.1 8 Reactome Database ID Release 81 10207335 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207335 Reactome R-SSC-70281 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70281.1 GENE ONTOLOGY GO:0003983 gene ontology term for cellular function MI MI:0355 Same Catalyst Activity Reactome Database ID Release 81 10207336 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207336 Reactome Database ID Release 81 10207338 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207338 Reactome R-SSC-70286 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70286.1 Cytosolic UDP-glucose pyrophosphorylase 2 (UGP2) catalyzes the reaction of UTP and glucose 1-phosphate to form UDP glucose and pyrophosphate (Knop and Hansen 1970; Duggleby et al. 1996). UGP2 is inferred to occur in the cell as a homooctamer from studies of its bovine homologue (Levine et al. 1969). 8631325 Pubmed 1996 Sequence differences between human muscle and liver cDNAs for UDPglucose pyrophosphorylase and kinetic properties of the recombinant enzymes expressed in Escherichia coli. Duggleby, RG Chao, YC Huang, JG Peng, HL Chang, HY Eur J Biochem 235:173-9 5427280 Pubmed 1970 Uridine diphosphate glucose pyrophosphorylase. IV. Crystallization and properties of the enzyme from human liver Knop, JK Hansen, RG J Biol Chem 245:2499-504 5348606 Pubmed 1969 Uridine diphosphate glucose pyrophosphorylase. II Levine, S Gillett, TA Hageman, E Hansen, RG J Biol Chem 244:5729-34 inferred from electronic annotation EVIDENCE CODE ECO:0000203 LEFT-TO-RIGHT 2.4.1.186 Autoglucosylation of GYG1 complexed with GYS1-a 8 UDP-glucose + GYG1 => 8 UDP + oligoGlc-GYG1 (GYS1-a) 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> GYG1:GYS1-a tetramer Reactome DB_ID: 10266130 GYS1 GYS1 F1RIP1 Reactome DB_ID: 10266061 UniProt:F1RIP1 GYS1 UniProt F1RIP1 2 EQUAL 737 EQUAL Reactome Database ID Release 81 10266061 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266061 Reactome R-SSC-71565 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71565.1 2 Mg2+ Magnesium magnesium(2+) Mg++ Reactome DB_ID: 29926 magnesium(2+) [ChEBI:18420] magnesium(2+) ChEBI CHEBI:18420 Reactome Database ID Release 81 29926 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29926 Reactome R-ALL-29926 5 Reactome stable identifier. Use this 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-29926.5 COMPOUND C00305 1 GYG1 GYG1 A0A286ZUI9 Reactome DB_ID: 10266073 UniProt:A0A286ZUI9 GYG1 UniProt A0A286ZUI9 2 EQUAL 350 EQUAL Reactome Database ID Release 81 10266073 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266073 Reactome R-SSC-70190 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70190.1 2 Reactome Database ID Release 81 10266130 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266130 Reactome R-SSC-3322050 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322050.1 8 UDP uridine 5'-diphosphate UDP(3-) Reactome DB_ID: 110096 UDP(3-) [ChEBI:58223] UDP(3-) 5'-O-[(phosphonatooxy)phosphinato]uridine UDP uridine 5'-diphosphate trianion uridine 5'-diphosphate(3-) UDP trianion uridine 5'-diphosphate ChEBI CHEBI:58223 Reactome Database ID Release 81 110096 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=110096 Reactome R-ALL-110096 3 Reactome stable identifier. Use this 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-110096.3 COMPOUND C00015 8 oligoGlc-GYG1:GYS1-a tetramer Reactome DB_ID: 10266066 2 GYG1 oligo((1,4)-alpha-glucosyl) GYG1 A0A286ZUI9 Reactome DB_ID: 10266064 195 EQUAL O4'-glucosyl-L-tyrosine MOD MOD:00166 2 EQUAL 350 EQUAL Reactome Database ID Release 81 10266064 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266064 Reactome R-SSC-70292 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70292.1 2 1 Reactome Database ID Release 81 10266066 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266066 Reactome R-SSC-3322035 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322035.1 ACTIVATION activeUnit: #Protein3 GENE ONTOLOGY GO:0008466 Reactome Database ID Release 81 10266131 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266131 Reactome Database ID Release 81 10266133 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266133 Reactome R-SSC-3322025 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322025.1 Glycogenin 1 (GYG1) catalyzes its autoglycosylation reaction with UDP-glucose to form oligo (1,4)-alpha-D-glucosyl GYG1 (Moslemi et al. 2010). The oligosaccharide is annotated here as containing four glucose residues. Glycogenin occurs as a homodimer complexed with two molecules of glycogen synthase 1 (GYS1), here in an unphosphorylated (a) form (Roach et al. 2012). 22248338 Pubmed 2012 Glycogen and its metabolism: some new developments and old themes Roach, Peter J DePaoli-Roach, Anna A Hurley, Thomas D Tagliabracci, Vincent S Biochem. J. 441:763-87 20357282 Pubmed 2010 Glycogenin-1 deficiency and inactivated priming of glycogen synthesis Moslemi, Ali-Reza Lindberg, Christopher Nilsson, Johanna Tajsharghi, Homa Andersson, Bert Oldfors, A N. Engl. J. Med. 362:1203-10 LEFT-TO-RIGHT 2.4.1.186 Autoglucosylation of GYG1 complexed with GYS1-b 8 UDP-glucose + GYG1 => 8 UDP + oligoGlc-GYG1 (GYS1-b) 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> 8 GYG1:GYS1-b tetramer Reactome DB_ID: 10266078 1 F1RIP1 phospho-GYS1 p-S-GYS1 Reactome DB_ID: 10266076 O-phospho-L-serine MOD MOD:00046 1 EQUAL 737 EQUAL Reactome Database ID Release 81 10266076 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266076 Reactome R-SSC-71570 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71570.1 2 2 Reactome Database ID Release 81 10266078 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266078 Reactome R-SSC-3322062 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322062.1 8 oligoGlc-GYG1:GYS1-b tetramer Reactome DB_ID: 10266080 2 1 2 Reactome Database ID Release 81 10266080 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266080 Reactome R-SSC-3322053 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322053.1 ACTIVATION activeUnit: #Protein3 Reactome Database ID Release 81 10266081 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266081 Reactome Database ID Release 81 10266083 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266083 Reactome R-SSC-3322003 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322003.1 Glycogenin 1 (GYG1) catalyzes its autoglycosylation reaction with UDP-glucose to form oligo (1,4)-alpha-D-glucosyl GYG1 (Moslemi et al. 2010). The oligosaccharide is annotated here as containing four glucose residues. Glycogenin occurs as a homodimer complexed with two molecules of glycogen synthase 1 (GYS1), here in a phosphorylated (b) form (Roach et al. 2012). LEFT-TO-RIGHT 2.4.1.186 Autoglucosylation of GYG2 complexed with GYS2-a 8 UDP-glucose + GYG2 => 8 UDP + oligoGlc-GYG2 (GYS2-a) 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> 8 GYG2:GYS2-a tetramer Reactome DB_ID: 10266111 1 GYG2 GYG2 A0A5G2R2E9 Reactome DB_ID: 10239627 UniProt:A0A5G2R2E9 GYG2 UniProt A0A5G2R2E9 1 EQUAL 501 EQUAL Reactome Database ID Release 81 10239627 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10239627 Reactome R-SSC-70205 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70205.1 2 GYS2 GYS2 A0A287B5W1 Reactome DB_ID: 10266099 UniProt:A0A287B5W1 GYS2 UniProt A0A287B5W1 1 EQUAL 703 EQUAL Reactome Database ID Release 81 10266099 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266099 Reactome R-SSC-71599 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71599.1 2 Reactome Database ID Release 81 10266111 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266111 Reactome R-SSC-3322031 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322031.1 8 oligoGlc-GYG2:GYS2-a tetramer Reactome DB_ID: 10266104 1 GYG2 oligo((1,4)-alpha-glycosyl) GYG2 A0A5G2R2E9 Reactome DB_ID: 10266102 228 EQUAL 1 EQUAL 501 EQUAL Reactome Database ID Release 81 10266102 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266102 Reactome R-SSC-70289 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70289.1 2 2 Reactome Database ID Release 81 10266104 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266104 Reactome R-SSC-3321999 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3321999.1 ACTIVATION activeUnit: #Protein6 Reactome Database ID Release 81 10266112 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266112 Reactome Database ID Release 81 10266114 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266114 Reactome R-SSC-3322014 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322014.1 Glycogenin 2 (GYG2) catalyzes its autoglycosylation reaction with UDP-glucose to form oligo (1,4)-alpha-D-glucosyl GYG2 (Mu et al. 1997). The oligosaccharide is annotated here as containing four glucose residues. Glycogenin occurs as a homodimer complexed with two molecules of glycogen synthase 1 (GYS2), here in a unphosphorylated (a) form (Roach et al. 2012). 9346895 Pubmed 1997 Glycogenin-2, a novel self-glucosylating protein involved in liver glycogen biosynthesis. Mu, J Skurat, AV Roach, Peter J J Biol Chem 272:27589-97 LEFT-TO-RIGHT 2.4.1.186 Autoglucosylation of GYG2 complexed with GYS2-b 8 UDP-glucose + GYG2 => 8 UDP + oligoGlc-GYG2 (GYS2-b) 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> GYG2:GYS2-b tetramer Reactome DB_ID: 10266123 1 2 p-S-GYS2 phospho-GYS2 A0A287B5W1 Reactome DB_ID: 10266121 1 EQUAL 703 EQUAL Reactome Database ID Release 81 10266121 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266121 Reactome R-SSC-71604 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71604.1 2 Reactome Database ID Release 81 10266123 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266123 Reactome R-SSC-3322065 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322065.1 8 oligoGlc-GYG2:GYS2-b tetramer Reactome DB_ID: 10266125 1 2 2 Reactome Database ID Release 81 10266125 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266125 Reactome R-SSC-3322007 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322007.1 8 ACTIVATION activeUnit: #Protein6 Reactome Database ID Release 81 10266126 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266126 Reactome Database ID Release 81 10266128 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266128 Reactome R-SSC-3322019 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322019.1 Glycogenin 2 (GYG2) catalyzes its autoglycosylation reaction with UDP-glucose to form oligo (1,4)-alpha-D-glucosyl GYG2 (Mu et al. 1997). The oligosaccharide is annotated here as containing four glucose residues. Glycogenin occurs as a homodimer complexed with two molecules of glycogen synthase 1 (GYS2), here in a phosphorylated (b) form (Roach et al. 2012). LEFT-TO-RIGHT 2.4.1.11 GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1 8 UDP-glucose + ((1,4)-alpha-D-glucosyl)4 glycogenin2 => 8 UDP + ((1,4)-alpha-D-glucosyl)8 glycogenin2 (GYS1-a) 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> 8 polyGlc-GYG1:GYS1-a tetramer Reactome DB_ID: 10266068 2 1 GYG1 poly((1,4)-alpha-glucosyl)GYG1 A0A286ZUI9 Reactome DB_ID: 10208574 195 EQUAL 2 EQUAL 350 EQUAL Reactome Database ID Release 81 10208574 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208574 Reactome R-SSC-70186 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70186.1 2 Reactome Database ID Release 81 10266068 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266068 Reactome R-SSC-3322054 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322054.1 8 ACTIVATION activeUnit: #Protein2 GENE ONTOLOGY GO:0004373 Reactome Database ID Release 81 10266069 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266069 Reactome Database ID Release 81 10266071 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266071 Reactome R-SSC-3322001 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322001.1 Unphosphorylated glycogen synthase 1 (GYS1-a) complexed with oligo-D-glucose-GYG1 catalyzes the polyglucosylation of the latter. (Here the addition of four glucose residues is annotated.) (Cameron et al. 2009; Roach et al. 2012). 19699667 Pubmed 2009 Identification of a novel mutation in GYS1 (muscle-specific glycogen synthase) resulting in sudden cardiac death, that is diagnosable from skin fibroblasts Cameron, JM Levandovskiy, V Mackay, Nevena Utgikar, Rucha Ackerley, Cameron A Chiasson, David Halliday, William Raiman, Julian Robinson, BH Mol. Genet. Metab. 98:378-82 LEFT-TO-RIGHT 2.4.1.11 Phosphorylated GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1 8 UDP-glucose + ((1,4)-alpha-D-glucosyl)4 glycogenin => 8 UDP + ((1,4)-alpha-D-glucosyl)8 glycogenin (GYS1-b) 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> 8 8 polyGlc-GYG1:GYS1-b tetramer Reactome DB_ID: 10266135 1 2 2 Reactome Database ID Release 81 10266135 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266135 Reactome R-SSC-3322063 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322063.1 ACTIVATION activeUnit: #Protein5 Reactome Database ID Release 81 10266136 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266136 Reactome Database ID Release 81 10266138 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266138 Reactome R-SSC-3322041 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322041.1 Phosphorylated glycogen synthase 1 (GYS1-b) complexed with oligo-D-glucose-GYG1 catalyzes the polyglucosylation of the latter. (Here the addition of four glucose residues is annotated.) This reaction is allosterically activated by glucose-6-phosphate (G6P) (Cameron et al. 2009; Roach et al. 2012). Glucose-6-phosphate (G6P) activates the phosphorylated ("b") form of GYS1 ("muscle") glycogen synthase (Roach et al. 2012). ACTIVATION Reactome Database ID Release 81 3322037 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=3322037 Reactome R-HSA-3322037 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-3322037.1 G6P alpha-D-glucose 6-phosphate alpha-D-glucose 6-phosphate(2-) Reactome DB_ID: 30537 alpha-D-glucose 6-phosphate(2-) [ChEBI:58225] alpha-D-glucose 6-phosphate(2-) alpha-D-glucopyranose 6-phosphate alpha-D-glucose 6-phosphate dianion alpha-D-glucose 6-phosphate 6-O-phosphonato-alpha-D-glucopyranose ChEBI CHEBI:58225 Reactome Database ID Release 81 30537 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=30537 Reactome R-ALL-30537 4 Reactome stable identifier. Use this 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-30537.4 COMPOUND C00668 LEFT-TO-RIGHT 2.4.1.11 GYS2 catalyzes the polyglucosylation of oligoGlc-GYG2 8 UDP-glucose + ((1,4)-alpha-D-glucosyl)4 glycogenin2 => 8 UDP + ((1,4)-alpha-D-glucosyl)8 glycogenin2 (GYS2-a) 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> 8 8 polyGlc-GYG2:GYS2-a tetramer Reactome DB_ID: 10266106 1 GYG2 poly((1,4)-alpha-glucosyl)GYG2 A0A5G2R2E9 Reactome DB_ID: 10208579 228 EQUAL 1 EQUAL 501 EQUAL Reactome Database ID Release 81 10208579 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208579 Reactome R-SSC-70188 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70188.1 2 2 Reactome Database ID Release 81 10266106 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266106 Reactome R-SSC-3322056 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322056.1 ACTIVATION activeUnit: #Protein7 Reactome Database ID Release 81 10266107 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266107 Reactome Database ID Release 81 10266109 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266109 Reactome R-SSC-3322009 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322009.1 Unphosphorylated glycogen synthase 2 (GYS2-a) complexed with oligo-D-glucose-GYG2 catalyzes the polyglucosylation of the latter. (Here the addition of four glucose residues is annotated.) (Orho et al. 1998; Roach et al. 2012). 9691087 Pubmed 1998 Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0 Orho, Marju Bosshard, Nils U Buist, NR Gitzelmann, Richard Aynsley-Green, A Blumel, P Gannon, MC Nuttall, FQ Groop, LC J Clin Invest 102:507-15 LEFT-TO-RIGHT 2.4.1.18 GBE1 catalyzes branch formation in polyGlc-GYG1 complexed with GYS1-a poly((1,4)-alpha-D-glucosyl) glycogenin1 => glycogen-glycogenin 1 (GYS1-a) 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> glycogen-GYG1:GYS1-a tetramer Reactome DB_ID: 10266088 2 1 GYG1 glycogen-GYG1 A0A286ZUI9 Reactome DB_ID: 10266086 195 EQUAL 2 EQUAL 350 EQUAL Reactome Database ID Release 81 10266086 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266086 Reactome R-SSC-70174 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70174.1 2 Reactome Database ID Release 81 10266088 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266088 Reactome R-SSC-3322059 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322059.1 ACTIVATION GBE1 GBE1 A0A480PP98 Reactome DB_ID: 10266092 UniProt:A0A480PP98 GBE1 UniProt A0A480PP98 1 EQUAL 702 EQUAL Reactome Database ID Release 81 10266092 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266092 Reactome R-SSC-71576 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71576.1 GENE ONTOLOGY GO:0003844 Reactome Database ID Release 81 10266093 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266093 Reactome Database ID Release 81 10266095 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266095 Reactome R-SSC-3322005 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322005.1 Cytosolic glycogen branching enzyme (GBE1) associated with glycogen granules transfers terminal alpha(1,4) glucose blocks to form alpha(1,6) branches on growing glycogen molecules formed on glycogenin 1 (GYG1) complexed with unphosphorylated glycogen synthase 1 (GYS1-a) (Bao et al. 1996; Roach et al. 2012). 8613547 Pubmed 1996 Hepatic and neuromuscular forms of glycogen storage disease type IV caused by mutations in the same glycogen-branching enzyme gene. Bao, Y Kishnani, Priya S Wu, Jer-Yuarn Chen, Yuan-Tsong J Clin Invest 97:941-8 LEFT-TO-RIGHT 2.4.1.18 GBE1 catalyzes branch formation in polyGlc-GYG1 complexed with GYS1-b poly((1,4)-alpha-D-glucosyl) glycogenin1 => glycogen-glycogenin 1 (GYS1-b) 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> glycogen-GYG1:GYS1-b tetramer Reactome DB_ID: 10266140 1 2 2 Reactome Database ID Release 81 10266140 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266140 Reactome R-SSC-3322021 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322021.1 ACTIVATION Reactome Database ID Release 81 10266142 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266142 Reactome R-SSC-3322057 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322057.1 Cytosolic glycogen branching enzyme (GBE1) associated with glycogen granules transfers terminal alpha(1,4) glucose blocks to form alpha(1,6) branches on growing glycogen molecules formed on glycogenin 1 (GYG1) complexed with phosphorylated glycogen synthase 1 (GYS1-b) (Bao et al. 1996; Roach et al. 2012). LEFT-TO-RIGHT 2.4.1.18 GBE1 catalyzes branch formation in polyGlc-GYG2 complexed with GYS2-a poly((1,4)-alpha-D-glucosyl) glycogenin2 => glycogen-glycogenin 2 (GYS2-a) 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> glycogen-GYG2:GYS2-a tetramer Reactome DB_ID: 10266116 1 2 GYG2 glycogen-GYG2 A0A5G2R2E9 Reactome DB_ID: 10208566 228 EQUAL 1 EQUAL 501 EQUAL Reactome Database ID Release 81 10208566 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208566 Reactome R-SSC-70176 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70176.1 2 Reactome Database ID Release 81 10266116 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266116 Reactome R-SSC-3322011 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322011.1 ACTIVATION Reactome Database ID Release 81 10266118 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266118 Reactome R-SSC-3322016 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322016.1 Cytosolic glycogen branching enzyme (GBE1) associated with glycogen granules transfers terminal alpha(1,4) glucose blocks to form alpha(1,6) branches on growing glycogen molecules formed on glycogenin 2 (GYG2) complexed with unphosphorylated glycogen synthase 2 (GYS2-a) (Bao et al. 1996; Roach et al. 2012). LEFT-TO-RIGHT PPP1R3C binds to glycogen:GYG1:GYS1 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> F1SCX0 PPP1R3C PPP1R3C Reactome DB_ID: 10266762 UniProt:F1SCX0 PPP1R3C UniProt F1SCX0 1 EQUAL 317 EQUAL Reactome Database ID Release 81 10266762 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266762 Reactome R-SSC-3777102 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3777102.1 PPP1R3C:glycogen-GYG1:GYS1-a tetramer Reactome DB_ID: 10266802 2 1 2 1 Reactome Database ID Release 81 10266802 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266802 Reactome R-SSC-3777096 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3777096.1 Reactome Database ID Release 81 10266804 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266804 Reactome R-SSC-3781023 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3781023.1 Protein phosphatase 1 regulatory subunit 3C (PPP1R3C, PTG) binds to glycogen particles containing GYG1 (glycogenin 1) and GYS1 (glycogen synthase 1). PPP1R3C appears to function as a scaffolding protein to mediate association of other proteins with glycogen granules and to promote the synthesis of glycogen (Worby et al. 2008). 18070875 Pubmed 2008 Malin decreases glycogen accumulation by promoting the degradation of protein targeting to glycogen (PTG) Worby, Carolyn A Gentry, Matthew S Dixon, JE J. Biol. Chem. 283:4069-76 LEFT-TO-RIGHT PPP1R3C binds to glycogen:GYG2:GYS2 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> PPP1R3C:glycogen-GYG2:GYS2-a tetramer Reactome DB_ID: 10266764 1 2 1 2 Reactome Database ID Release 81 10266764 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266764 Reactome R-SSC-3777104 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3777104.1 Reactome Database ID Release 81 10266783 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266783 Reactome R-SSC-3780997 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3780997.1 Protein phosphatase 1 regulatory subunit 3C (PPP1R3C, PTG) binds to glycogen particles containing GYG2 (glycogenin 2) and GYS2 (glycogen synthase 2). PPP1R3C appears to function as a scaffolding protein to mediate association of other proteins with glycogen granules and to promote the synthesis of glycogen (Worby et al. 2008). This reaction, involving GYG and GYS isoforms largely expressed in the liver has not been studied in detail but is inferred from the better-characterized one involving GYG1- and GYS1-containing glycogen particles ("muscle" isoforms, widely expressed in the body outside the liver). LEFT-TO-RIGHT GYS1 catalyzes the incorporation of phosphoglucose into glycogen-GYG1 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> UMP uridine 5'-monophosphate uridylic acid Uridine monophosphate 5'Uridylic acid uridine 5'-monophosphate(2-) Reactome DB_ID: 109402 uridine 5'-monophosphate(2-) [ChEBI:57865] uridine 5'-monophosphate(2-) uridine 5'-phosphate 5'-O-phosphonatouridine UMP dianion UMP UMP(2-) 5'-uridylate ChEBI CHEBI:57865 Reactome Database ID Release 81 109402 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=109402 Reactome R-ALL-109402 5 Reactome stable identifier. Use this 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-109402.5 COMPOUND C00105 PPP1R3C:phosphoglycogen-GYG1:GYS1-a tetramer Reactome DB_ID: 10266788 2 1 GYG1 polysaccharide-P-GYG1 A0A286ZUI9 Reactome DB_ID: 10266786 195 EQUAL 2 EQUAL 350 EQUAL Reactome Database ID Release 81 10266786 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266786 Reactome R-SSC-3777092 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3777092.1 2 1 Reactome Database ID Release 81 10266788 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266788 Reactome R-SSC-3777093 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3777093.1 ACTIVATION activeUnit: #Protein2 GENE ONTOLOGY GO:0061547 Reactome Database ID Release 81 10266805 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266805 Reactome Database ID Release 81 10266807 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266807 Reactome R-SSC-3781024 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3781024.1 Glycogen synthase 1 (GYS1) catalyzes the incorporation of phosphoglucose into the glycogen-GYG1 molecules with which it is associated in a cytosolic glycogen granule. This reaction occurs at a low rate, yielding approximately one molecule of glucose phosphorylated at its C2, C3, or C6 positions incorporated into a growing glycogen polymer per ten thousand glucose molecules incorporated (DePaoli-Roach et al. 2015; Irimia et al. 2015; Nitschke et al. 2013; Tagliabracci et al. 2011). The function of these small amounts of phosphoglucose in normal glycogen remains to be established. 26216881 Pubmed 2015 Muscle Glycogen Remodeling and Glycogen Phosphate Metabolism following Exhaustive Exercise of Wild Type and Laforin Knockout Mice Irimia, Jose M Tagliabracci, Vincent S Meyer, Catalina M Segvich, Dyann M DePaoli-Roach, Anna A Roach, Peter J J. Biol. Chem. 290:22686-98 25416783 Pubmed 2015 Glycogen phosphomonoester distribution in mouse models of the progressive myoclonic epilepsy, Lafora disease DePaoli-Roach, Anna A Contreras, Christopher J Segvich, Dyann M Heiss, Christian Ishihara, Mayumi Azadi, Parastoo Roach, Peter J J. Biol. Chem. 290:841-50 21356517 Pubmed 2011 Phosphate incorporation during glycogen synthesis and Lafora disease Tagliabracci, Vincent S Heiss, Christian Karthik, Chandra Contreras, Christopher J Glushka, John Ishihara, Mayumi Azadi, Parastoo Hurley, Thomas D DePaoli-Roach, Anna A Roach, Peter J Cell Metab. 13:274-82 23663739 Pubmed 2013 Hyperphosphorylation of glucosyl C6 carbons and altered structure of glycogen in the neurodegenerative epilepsy Lafora disease Nitschke, Felix Wang, Peixiang Schmieder, Peter Girard, Jean-Marie Awrey, Donald E Wang, Tony Israelian, Johan Zhao, Xiaochu Turnbull, Julie Heydenreich, Matthias Kleinpeter, Erich Steup, Martin Minassian, Berge A Cell Metab. 17:756-67 LEFT-TO-RIGHT GYS2 catalyzes the incorporation of phosphoglucose into glycogen-GYG2 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> PPP1R3C:phosphoglycogen-GYG2:GYS2-a tetramer Reactome DB_ID: 10266769 1 GYG2 polysaccharide-P-GYG2 A0A5G2R2E9 Reactome DB_ID: 10266767 228 EQUAL 1 EQUAL 501 EQUAL Reactome Database ID Release 81 10266767 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266767 Reactome R-SSC-3777097 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3777097.1 2 2 1 Reactome Database ID Release 81 10266769 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266769 Reactome R-SSC-3777094 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3777094.1 ACTIVATION activeUnit: #Protein7 Reactome Database ID Release 81 10266770 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266770 Reactome Database ID Release 81 10266772 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266772 Reactome R-SSC-3780994 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3780994.1 Glycogen synthase 2 (GYS2) catalyzes the incorporation of phosphoglucose into the glycogen-GYG1 molecules with which it is associated in a cytosolic glycogen granule. This reaction occurs at a low rate, yielding approximately one molecule of glucose phosphorylated at its C2, C3, or C6 positions incorporated into a growing glycogen polymer per ten thousand glucose molecules incorporated (DePaoli-Roach et al. 2015; Irimia et al. 2015; Nitschke et al. 2013; Tagliabracci et al. 2011). The function of these small amounts of phosphoglucose in normal glycogen remains to be established. This reaction has been characterized in muscle cells, where it is catalyzed by the homologous GYS1 enzyme. The occurrence of the reaction in liver, catalyzed by GYS2, can be inferred from the fact that in the absence of the enzyme EMP2A (laforin) that removes these phosphate groups, abnormally phosphorylated glycogen accumulates in both tissues (Worby et al. 2008). LEFT-TO-RIGHT EPM2A dimer dephosphorylates phosphoglycogen-GYG1 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> H2O water Reactome DB_ID: 29356 water [ChEBI:15377] water ChEBI CHEBI:15377 Reactome Database ID Release 81 29356 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29356 Reactome R-ALL-29356 5 Reactome stable identifier. Use this 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-29356.5 COMPOUND C00001 EPM2A:PPP1R3C:phosphoglycogen-GYG1:GYS1-a tetramer Reactome DB_ID: 10266797 2 1 2 1 EPM2A dimer Reactome DB_ID: 10266776 Ghost homologue of EPM2A Reactome DB_ID: 10266774 Reactome Database ID Release 81 10266774 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266774 Reactome R-SSC-3777103 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3777103.1 2 Reactome Database ID Release 81 10266776 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266776 Reactome R-SSC-6805697 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-6805697.1 1 Reactome Database ID Release 81 10266797 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266797 Reactome R-SSC-3780999 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3780999.1 Pi Orthophosphate hydrogenphosphate Phosphate Inorganic Phosphate Reactome DB_ID: 29372 hydrogenphosphate [ChEBI:43474] hydrogenphosphate hydrogen phosphate phosphate [PO3(OH)](2-) INORGANIC PHOSPHATE GROUP HYDROGENPHOSPHATE ION HPO4(2-) [P(OH)O3](2-) ChEBI CHEBI:43474 Reactome Database ID Release 81 29372 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29372 Reactome R-ALL-29372 4 Reactome stable identifier. Use this 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-29372.4 COMPOUND C00009 EPM2A:PPP1R3C:glycogen-GYG1:GYS1-a tetramer Reactome DB_ID: 10266790 2 1 2 1 1 Reactome Database ID Release 81 10266790 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266790 Reactome R-SSC-3781000 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3781000.1 ACTIVATION GENE ONTOLOGY GO:0019203 Reactome Database ID Release 81 10266798 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266798 Reactome Database ID Release 81 10266800 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266800 Reactome R-SSC-3781018 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3781018.1 EPM2A (laforin), associated with PPP1R3C (protein phosphatase 1 regulatory subunit 3C, PTG) and phosphoglycogen-GYG1 in a cytosolic glycogen particle, catalyzes the dephosphorylation of phosphoglycogen (Tagliabracci et al. 2007). The catalytically active form of EPM2A has been shown to be a homodimer (Raththagala et al. 2015; Sankhala et al. 2015). 18040046 Pubmed 2007 Laforin is a glycogen phosphatase, deficiency of which leads to elevated phosphorylation of glycogen in vivo Tagliabracci, Vincent S Turnbull, Julie Wang, Wei Girard, Jean-Marie Zhao, Xiaochu Skurat, AV Delgado-Escueta, Antonio V Minassian, Berge A DePaoli-Roach, Anna A Roach, Peter J Proc. Natl. Acad. Sci. U.S.A. 104:19262-6 25538239 Pubmed 2015 Dimeric quaternary structure of human laforin Sankhala, Rajeshwer S Koksal, Adem C Ho, Lan Nitschke, Felix Minassian, Berge A Cingolani, Gino J. Biol. Chem. 290:4552-9 25544560 Pubmed 2015 Structural mechanism of laforin function in glycogen dephosphorylation and lafora disease Raththagala, Madushi Brewer, M Kathryn Parker, Matthew W Sherwood, Amanda R Wong, Brian K Hsu, Simon Bridges, Travis M Paasch, Bradley C Hellman, Lance M Husodo, Satrio Meekins, David A Taylor, Adam O Turner, Benjamin D Auger, Kyle D Dukhande, Vikas V Chakravarthy, Srinivas Sanz, Pascual Woods, Virgil L Li, Sheng Vander Kooi, Craig W Gentry, Matthew S Mol. Cell 57:261-72 LEFT-TO-RIGHT EPM2A dimer dephosphorylates phosphoglycogen-GYG2 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> EPM2A:PPP1R3C:phosphoglycogen-GYG2:GYS2-a tetramer Reactome DB_ID: 10266792 1 2 2 1 1 Reactome Database ID Release 81 10266792 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266792 Reactome R-SSC-3781017 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3781017.1 EPM2A:PPP1R3C:glycogen-GYG2:GYS2-a tetramer Reactome DB_ID: 10266778 1 2 1 2 1 Reactome Database ID Release 81 10266778 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266778 Reactome R-SSC-3780991 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3780991.1 ACTIVATION Reactome Database ID Release 81 10266793 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266793 Reactome Database ID Release 81 10266795 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10266795 Reactome R-SSC-3781011 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3781011.1 EPM2A (laforin), associated with PPP1R3C (protein phosphatase 1 regulatory subunit 3C, PTG) and phosphoglycogen-GYG2 in a cytosolic glycogen particle, catalyzes the dephosphorylation of phosphoglycogen (Tagliabracci et al. 2007). This reaction is inferred from the activity of EPM2A on phosphoglycogen-GYG1, and from the fact that in the absence of normal EPM2A activity, abnormally phosphorylated forms of both glycogen-GYG1 and glycogen-GYG2 accumulate in cells. The catalytically active form of EPM2A has been shown to be a homodimer (Raththagala et al. 2015; Sankhala et al. 2015). Reactome Database ID Release 81 10306291 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10306291 Reactome R-SSC-3322077 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3322077.1 GENE ONTOLOGY GO:0005978 gene ontology term for cellular process MI MI:0359 Glycogen, a highly branched glucose polymer, is formed and broken down in most human tissues, but is most abundant in liver and muscle, where it serves as a major stored fuel. Glycogen metabolism has been studied in most detail in muscle, although considerable experimental data are available concerning these reactions in liver as well. Glycogen metabolism in other tissues has not been studied as extensively, and is thought to resemble the muscle process. Glycogen synthesis involves five reactions. The first two, conversion of glucose 6-phosphate to glucose 1-phosphate and synthesis of UDP-glucose from glucose 1-phosphate and UTP, are shared with several other pathways. The next three reactions, the auto-catalyzed synthesis of a glucose oligomer on glycogenin, the linear extension of the glucose oligomer catalyzed by glycogen synthase, and the formation of branches catalyzed by glycogen branching enzyme, are unique to glycogen synthesis. Repetition of the last two reactions generates large, extensively branched glycogen polymers. The catalysis of glycogenin glucosylation and oligoglucose chain extension by distinct isozymes in liver and nonhepatic tissues allows them to be regulated independently (Agius 2008; Bollen et al. 1998; Roach et al. 2012). 18651836 Pubmed 2008 Glucokinase and molecular aspects of liver glycogen metabolism Agius, L Biochem. J. 414:1-18 9806880 Pubmed 1998 Specific features of glycogen metabolism in the liver Bollen, Matthieu Keppens, Stefaan Stalmans, Willy Biochem. J. 336:19-31 Glycogen breakdown (glycogenolysis) 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-RIGHT 2.7.11.19 glycogen phosphorylase (PYGL) dimer b + 2 ATP => glycogen phosphorylase (PYGL) dimer a + 2 ADP 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> PYGL dimer b form Reactome DB_ID: 10208539 PYGL PXLP-PYGL A0A287AL94 Reactome DB_ID: 10208537 UniProt:A0A287AL94 PYGL UniProt A0A287AL94 661 EQUAL N6-pyridoxal phosphate-L-lysine MOD MOD:00128 2 EQUAL 847 EQUAL Reactome Database ID Release 81 10208537 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208537 Reactome R-SSC-71580 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71580.1 2 Reactome Database ID Release 81 10208539 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208539 Reactome R-SSC-71581 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71581.1 ATP Adenosine 5'-triphosphate ATP(4-) Reactome DB_ID: 113592 ATP(4-) [ChEBI:30616] ATP(4-) ATP atp Adenosine 5'-triphosphate ChEBI CHEBI:30616 Reactome Database ID Release 81 113592 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113592 Reactome R-ALL-113592 5 Reactome stable identifier. Use this 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-113592.5 COMPOUND C00002 2 ADP Adenosine 5'-diphosphate ADP(3-) Reactome DB_ID: 29370 ADP(3-) [ChEBI:456216] ADP(3-) ADP 5&apos;-O-[(phosphonatooxy)phosphinato]adenosine ADP trianion ChEBI CHEBI:456216 Reactome Database ID Release 81 29370 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29370 Reactome R-ALL-29370 5 Reactome stable identifier. Use this 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-29370.5 COMPOUND C00008 2 PYGL dimer a form Reactome DB_ID: 10208544 A0A287AL94 phospho-PYGL PXLP-p-S15-PYGL Reactome DB_ID: 10208542 15 EQUAL 2 EQUAL 847 EQUAL Reactome Database ID Release 81 10208542 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208542 Reactome R-SSC-71585 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71585.1 2 Reactome Database ID Release 81 10208544 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208544 Reactome R-SSC-71586 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71586.1 ACTIVATION phosphorylase kinase complex (PHKL) Reactome DB_ID: 10208560 PHKG2 PHKG2 F1RG75 Reactome DB_ID: 10208556 UniProt:F1RG75 PHKG2 UniProt F1RG75 1 EQUAL 406 EQUAL Reactome Database ID Release 81 10208556 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208556 Reactome R-SSC-71533 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71533.1 4 Ghost homologue of CALM1 Reactome DB_ID: 10208558 Reactome Database ID Release 81 10208558 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208558 Reactome R-SSC-9016707 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-9016707.1 4 PHKA2 PHKA2 A0A287B6P0 Reactome DB_ID: 10208548 UniProt:A0A287B6P0 PHKA2 UniProt A0A287B6P0 1 EQUAL 1235 EQUAL Reactome Database ID Release 81 10208548 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208548 Reactome R-SSC-71524 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71524.1 4 PHKB PHKB A0A287AGE8 Reactome DB_ID: 10208552 UniProt:A0A287AGE8 PHKB UniProt A0A287AGE8 2 EQUAL 1093 EQUAL Reactome Database ID Release 81 10208552 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208552 Reactome R-SSC-71517 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71517.1 4 Reactome Database ID Release 81 10208560 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208560 Reactome R-SSC-71534 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71534.1 GENE ONTOLOGY GO:0004689 Reactome Database ID Release 81 10208561 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208561 Reactome Database ID Release 81 10208563 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208563 Reactome R-SSC-71588 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71588.1 The cytosolic phosphorylase kinase complex catalyzes the phosphorylation of glycogen phosphorylase (PYGL). Two forms of phosphorylase kinase complex have been described (Brushia and Walsh 1999). The one annotated here, consisting of four copies each of PHKA2 (alpha regulatory) (van den Berg et al. 1995), PHKB (beta regulatory) (Burwinkel et al. 2003a), PHKG2 (gamma catalytic) (Burwinkel et al. 2003b; Maichele et al. 2006) and CALM (calmodulin) subunits is abundant in liver and its action on the form of phosphorylase (PYGL) abundant in liver is described.<p>While initial studies of glycogen phosphorylase PGYM from rabbit muscle suggested that it is a homotetramer (Keller and Cori 1953), more recent work indicates that under physiological conditions the enzyme occurs as a homodimer (Huang and Graves 1970) and a dimeric structure for human PYGL enzyme is inferred here. 10487978 Pubmed 1999 Phosphorylase kinase: the complexity of its regulation is reflected in the complexity of its structure Brushia, RJ Walsh, DA Front Biosci 4:D618-41 8896567 Pubmed 1996 Mutations in the testis/liver isoform of the phosphorylase kinase gamma subunit (PHKG2) cause autosomal liver glycogenosis in the gsd rat and in humans Maichele, AJ Burwinkel, B Maire, I Sovik, O Kilimann, MW Nat Genet 14:337-40 5461220 Pubmed 1970 Correlation between subunit interactions and enzymatic activity of phosphorylase a. Method for determining equilibrium constants from initial rate measurements Huang, CY Graves, DJ Biochemistry 9:660-71 7847371 Pubmed 1995 X-linked liver phosphorylase kinase deficiency is associated with mutations in the human liver phosphorylase kinase alpha subunit. van den Berg, IE van Beurden, EA Malingré, HE van Amstel, HK Poll-The, BT Smeitink, JA Lamers, WH Berger, R Am J Hum Genet 56:381-7 12930917 Pubmed 2003 Severe phenotype of phosphorylase kinase-deficient liver glycogenosis with mutations in the PHKG2 gene Burwinkel, B Rootwelt, T Kvittingen, EA Chakraborty, PK Kilimann, MW Pediatr Res 54:834-9 13115432 Pubmed 1953 Enzymic conversion of phosphorylase a to phosphorylase b Keller, PJ Cori, GT Biochim Biophys Acta 12:235-8 12825073 Pubmed 2003 Muscle glycogenosis with low phosphorylase kinase activity: mutations in PHKA1, PHKG1 or six other candidate genes explain only a minority of cases Burwinkel, B Hu, B Schroers, A Clemens, PR Moses, SW Shin, YS Pongratz, D Vorgerd, M Kilimann, MW Eur J Hum Genet 11:516-26 ACTIVATION Reactome Database ID Release 81 2684957 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=2684957 Reactome R-HSA-2684957 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-2684957.1 Ca2+ Calcium calcium(2+) Reactome DB_ID: 74016 calcium(2+) [ChEBI:29108] calcium(2+) ChEBI CHEBI:29108 Reactome Database ID Release 81 74016 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=74016 Reactome R-ALL-74016 3 Reactome stable identifier. Use this 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-74016.3 COMPOUND C00076 LEFT-TO-RIGHT PYGM dimer, b form + 2 AMP <=> PYGM b dimer:AMP complex 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> PYGM dimer, b form Reactome DB_ID: 10208492 PYGM PXLP-PYGM A0A287B6I2 Reactome DB_ID: 10208490 UniProt:A0A287B6I2 PYGM UniProt A0A287B6I2 681 EQUAL 2 EQUAL 842 EQUAL Reactome Database ID Release 81 10208490 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208490 Reactome R-SSC-71537 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71537.1 2 Reactome Database ID Release 81 10208492 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208492 Reactome R-SSC-71538 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71538.1 AMP adenosine 5'-monophosphate adenosine 5'-monophosphate(2-) Adenylic acid Adenylate 5'-AMP 5'-Adenylic acid 5'-Adenosine monophosphate Reactome DB_ID: 76577 adenosine 5'-monophosphate(2-) [ChEBI:456215] adenosine 5'-monophosphate(2-) AMP Adenosine-5-monophosphate(2-) AMP dianion 5'-O-phosphonatoadenosine Adenosine-5-monophosphate dianion AMP(2-) ChEBI CHEBI:456215 Reactome Database ID Release 81 76577 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=76577 Reactome R-ALL-76577 5 Reactome stable identifier. Use this 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-76577.5 COMPOUND C00020 2 PYGM b dimer:AMP Reactome DB_ID: 10239623 2 2 Reactome Database ID Release 81 10239623 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10239623 Reactome R-SSC-453344 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453344.1 Reactome Database ID Release 81 10239633 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10239633 Reactome R-SSC-453342 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453342.1 Cytosolic, non-phosphorylated PGYM dimers (b form) can reversibly bind AMP. This reaction is negatively regulated by ATP and glucose 6-phosphate and is the means by which high levels of these molecules repress PGYM activity (Rath et al. 2000). 10949035 Pubmed 2000 Activation of human liver glycogen phosphorylase by alteration of the secondary structure and packing of the catalytic core Rath, Virginia L Ammirati, M LeMotte, PK Fennell, KF Mansour, MN Danley, DE Hynes, TR Schulte, GK Wasilko, DJ Pandit, J Mol Cell 6:139-48 INHIBITION Reactome Database ID Release 81 71507 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=71507 Reactome R-HSA-71507 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-71507.1 INHIBITION Reactome Database ID Release 81 453374 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=453374 Reactome R-HSA-453374 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-453374.1 LEFT-TO-RIGHT PYGM b dimer:AMP complex <=> PYGM dimer, b form + 2 AMP 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> 2 Reactome Database ID Release 81 10239625 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10239625 Reactome R-SSC-453338 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453338.1 Cytosolic, non-phosphorylated PGYM dimers (b form) complexed with AMP can reversibly dissociate (Rath et al. 2000). LEFT-TO-RIGHT glycogen-glycogenin-2 + n orthophosphate => limit dextrin-glycogenin-2 + n D-glucose 1-phosphate [PYGL] 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> glycogen-glycogenin-2 dimer Reactome DB_ID: 10208568 2 Mn2+ Manganese manganese(2+) Mn(II) Reactome DB_ID: 29418 manganese(2+) [ChEBI:29035] manganese(2+) Mn(2+) MANGANESE (II) ION manganese, ion (Mn2+) manganese(II) manganous ion ChEBI CHEBI:29035 Reactome Database ID Release 81 29418 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29418 Reactome R-ALL-29418 3 Reactome stable identifier. Use this 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-29418.3 COMPOUND C00034 1 Reactome Database ID Release 81 10208568 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208568 Reactome R-SSC-453240 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453240.1 limit dextrin-glycogenin-2 dimer Reactome DB_ID: 10208511 GYG2 limit dextrin-glycogenin-2 A0A5G2R2E9 Reactome DB_ID: 10208509 196 EQUAL 1 EQUAL 501 EQUAL Reactome Database ID Release 81 10208509 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208509 Reactome R-SSC-70181 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70181.1 2 1 Reactome Database ID Release 81 10208511 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208511 Reactome R-SSC-453330 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453330.1 ACTIVATION GENE ONTOLOGY GO:0008184 Reactome Database ID Release 81 10208569 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208569 Reactome Database ID Release 81 10208571 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208571 Reactome R-SSC-71590 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71590.1 The phosphorylated PYGL dimer (a form) of glycogen phosphorylase catalyzes the reaction of orthophosphate and glycogen-glycogenin 1 to form D-glucose 1-phosphate and limit dextrin-glycogenin 1. This reaction occurs on the surfaces of cytosolic glycogen granules. Non-phosphorylated PYGL dimers (b form) are catalytically inactive even in the presence of AMP. In the body, this reaction takes place in the liver where its dependence on hormonally stimulated PYGL phosphorylation (and lack of sensitivity to AMP) allow glucose mobilization in response to a demand for glucose from the rest of the body (Newgard et al. 1989; Rath et al. 2000). 2667896 Pubmed 1989 The family of glycogen phosphorylases: structure and function Newgard, CB Hwang, PK Fletterick, RJ Crit Rev Biochem Mol Biol 24:69-99 LEFT-TO-RIGHT 2.4.1.25 limit dextrin-glycogenin => ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl) glycogenin 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 Reactome limit dextrin-glycogenin dimer Reactome DB_ID: 10208513 limit dextrin-glycogenin-1 dimer Reactome DB_ID: 10208504 GYG1 limit dextrin-glycogenin-1 A0A286ZUI9 Reactome DB_ID: 10208502 194 EQUAL 2 EQUAL 350 EQUAL Reactome Database ID Release 81 10208502 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208502 Reactome R-SSC-70179 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70179.1 2 1 Reactome Database ID Release 81 10208504 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208504 Reactome R-SSC-453345 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453345.1 Reactome Database ID Release 81 10208513 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208513 Reactome R-SSC-453336 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453336.1 Converted from EntitySet in Reactome ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)GYG dimer ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl) glycogenin dimer Reactome DB_ID: 10208525 ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)GYG1 dimer Reactome DB_ID: 10208518 GYG1 ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)GYG1 A0A286ZUI9 Reactome DB_ID: 10208516 194 EQUAL 2 EQUAL 350 EQUAL Reactome Database ID Release 81 10208516 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208516 Reactome R-SSC-70298 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70298.1 2 1 Reactome Database ID Release 81 10208518 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208518 Reactome R-SSC-453360 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453360.1 ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)GYG2 dimer Reactome DB_ID: 10208523 GYG2 ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)GYG2 A0A5G2R2E9 Reactome DB_ID: 10208521 196 EQUAL 1 EQUAL 501 EQUAL Reactome Database ID Release 81 10208521 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208521 Reactome R-SSC-70300 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70300.1 2 1 Reactome Database ID Release 81 10208523 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208523 Reactome R-SSC-453365 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453365.1 Reactome Database ID Release 81 10208525 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208525 Reactome R-SSC-453350 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453350.1 ACTIVATION AGL AGL A0A287B3F4 Reactome DB_ID: 10208529 UniProt:A0A287B3F4 AGL UniProt A0A287B3F4 1 EQUAL 1532 EQUAL Reactome Database ID Release 81 10208529 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208529 Reactome R-SSC-71545 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71545.1 GENE ONTOLOGY GO:0004134 Reactome Database ID Release 81 10208530 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208530 Reactome Database ID Release 81 10208532 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208532 Reactome R-SSC-71552 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71552.1 Cytosolic debranching enzyme associated with glycogen granules transfers 3-glucose blocks from branches to the main linear polyglucose chain of limit dextrin formed on glycogenin 1 or glycogenin 2 (Chen et al. 1988; Shen et al. 1996). 8755644 Pubmed 1996 Mutations in exon 3 of the glycogen debranching enzyme gene are associated with glycogen storage disease type III that is differentially expressed in liver and muscle. Shen, Jinlong Bao, Y Liu, HM Lee, P Leonard, JV Chen, Yuan-Tsong J Clin Invest 98:352-7 2961257 Pubmed 1988 Glycogen debranching enzyme: purification, antibody characterization, and immunoblot analyses of type III glycogen storage disease. Chen, Yuan-Tsong He, JK Ding, JH Brown, Barbara Illingworth Am J Hum Genet 41:1002-15 LEFT-TO-RIGHT 3.2.1.33 ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)glycogenin => poly{(1,4)-alpha-glucosyl} glycogenin + alpha-D-glucose 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> Glc alpha-D-glucose Reactome DB_ID: 70113 alpha-D-glucose [ChEBI:17925] alpha-D-glucose ChEBI CHEBI:17925 Reactome Database ID Release 81 70113 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=70113 Reactome R-ALL-70113 3 Reactome stable identifier. Use this 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-70113.3 COMPOUND C00267 Converted from EntitySet in Reactome poly((1,4)-alpha-glucosyl)GYG dimer poly((1,4)-alpha-glucosyl) glycogenin dimer Reactome DB_ID: 10208583 poly((1,4)-alpha-glucosyl)GYG1 dimer Reactome DB_ID: 10208576 2 1 Reactome Database ID Release 81 10208576 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208576 Reactome R-SSC-453217 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453217.1 poly((1,4)-alpha-glucosyl)GYG2 dimer Reactome DB_ID: 10208581 2 1 Reactome Database ID Release 81 10208581 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208581 Reactome R-SSC-453216 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453216.1 Reactome Database ID Release 81 10208583 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208583 Reactome R-SSC-453224 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453224.1 ACTIVATION GENE ONTOLOGY GO:0004135 Reactome Database ID Release 81 10208584 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208584 Reactome Database ID Release 81 10208586 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208586 Reactome R-SSC-71593 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71593.1 At the beginning of this reaction, 1 molecule of '{(1,6)-alpha-glucosyl}poly{(1,4)-alpha-glucosyl}glycogenin-2' is present. At the end of this reaction, 1 molecule of 'alpha-D-glucose', and 1 molecule of 'poly{(1,4)-alpha-glucosyl}glycogenin-2' are present.<br><br> This reaction takes place in the 'cytoplasm' and is mediated by the 'amylo-alpha-1,6-glucosidase activity' of 'glycogen debranching enzyme'.<br> LEFT-TO-RIGHT poly((1,4)-alpha-glucosyl) glycogenin-2 + n orthophosphate => glycogenin-2 + n D-glucose 1-phosphate [PYGL] 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> GYG2 dimer Reactome DB_ID: 10239629 2 1 Reactome Database ID Release 81 10239629 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10239629 Reactome R-SSC-70214 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70214.1 ACTIVATION Reactome Database ID Release 81 10239631 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10239631 Reactome R-SSC-453339 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-453339.1 The phosphorylated PYGL dimer (a form) of glycogen phosphorylase catalyzes the reaction of orthophosphate and poly((1,4)-alpha-glucosyl) glycogenin-2 to form D-glucose 1-phosphate and glycogenin-2. This reaction occurs on the surfaces of cytosolic glycogen granules. Non-phosphorylated PYGL dimers (b form) are catalytically inactive even in the presence of AMP. In the body, this reaction takes place in the liver where its dependence on hormonally stimulated PYGL phosphorylation (and lack of sensitivity to AMP) allow glucose mobilization in response to a demand for glucose from the rest of the body (Newgard et al. 1989; Rath et al. 2000). LEFT-TO-RIGHT 3.2.1.20 GAA hydrolyzes lysosomal glycogen GAA hydrolyzes alpha(1,6) linkages in lysosomal glycogen GAA hydrolyzes alpha(1,4) linkages in lysosomal glycogen 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> H2O water Reactome DB_ID: 1605715 lysosomal lumen GENE ONTOLOGY GO:0043202 Reactome Database ID Release 81 1605715 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1605715 Reactome R-ALL-1605715 3 Reactome stable identifier. Use this 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-1605715.3 COMPOUND C00001 2 glycogen-GYG1 dimer Reactome DB_ID: 10301847 GYG1 glycogen-GYG1 A0A286ZUI9 Reactome DB_ID: 10301845 2 EQUAL 350 EQUAL Reactome Database ID Release 81 10301845 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10301845 Reactome R-SSC-9036728 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-9036728.1 2 Mn2+ Manganese manganese(2+) Mn(II) Reactome DB_ID: 5357557 Reactome Database ID Release 81 5357557 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=5357557 Reactome R-ALL-5357557 3 Reactome stable identifier. Use this 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-5357557.3 COMPOUND C00034 1 Reactome Database ID Release 81 10301847 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10301847 Reactome R-SSC-9036726 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-9036726.1 Glc alpha-D-glucose Reactome DB_ID: 1605745 Reactome Database ID Release 81 1605745 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1605745 Reactome R-ALL-1605745 3 Reactome stable identifier. Use this 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-1605745.3 COMPOUND C00267 2 poly((1,4)-alpha-glucosyl)GYG1 dimer Reactome DB_ID: 10301851 GYG1 poly((1,4)-alpha-glucosyl)GYG1 A0A286ZUI9 Reactome DB_ID: 10301849 2 EQUAL 350 EQUAL Reactome Database ID Release 81 10301849 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10301849 Reactome R-SSC-5357545 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-5357545.1 2 1 Reactome Database ID Release 81 10301851 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10301851 Reactome R-SSC-5357533 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-5357533.1 ACTIVATION GAA GAA (123-?) F1RZ82 Reactome DB_ID: 10301853 UniProt:F1RZ82 GAA UniProt F1RZ82 123 EQUAL -1 EQUAL Reactome Database ID Release 81 10301853 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10301853 Reactome R-SSC-5357560 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-5357560.1 GENE ONTOLOGY GO:0090599 Reactome Database ID Release 81 10301856 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10301856 Reactome Database ID Release 81 10301858 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10301858 Reactome R-SSC-9036727 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-9036727.1 While most glycogen is cytosolic, glycogen granules are also found in lysosomes, which they are thought to enter by autophagy. Once in lysosomes, the polysaccharide component of the granules is degraded by a single enzyme, lysosomal alpha-glucosidase (GAA), which hydrolyzes both alpha(1,4) and alpha(1,6) linkages to yield free glucose (Brown et al. 1970). The fate of the glycogenin protein component of the granules has not been studied; it is thought to be degraded by the diverse lysosomal proteases (Muller et al. 2012).<br>Mutations that reduce the activity of GAA are associated with glycogen storage disease type II (Pompe disease) (Hirschhorn & Reuser 2001; Leslie & Tinkle). The most active forms of GAA are 70 and 76 kDa polypeptides generated by removal of both aminoterminal and carboxyterminal fragments (Brown et al. 1970; Hoefsloot et al. 1988; Wisselaar et al. 1993). 21767668 Pubmed 2012 Specific functions of lysosomal proteases in endocytic and autophagic pathways Müller, Sabrina Dennemärker, Julia Reinheckel, Thomas Biochim. Biophys. Acta 1824:34-43 8420990 Pubmed 1993 Structural and functional changes of lysosomal acid alpha-glucosidase during intracellular transport and maturation Wisselaar, Heleen A Kroos, Marian A Hermans, Monique M P van Beeumen, Jos Reuser, Arnold J J J. Biol. Chem. 268:2223-31 5264799 Pubmed 1970 Simultaneous absence of alpha-1,4-glucosidase and alpha-1,6-glucosidase activities (pH 4) in tissues of children with type II glycogen storage disease Brown, Barbara Illingworth Brown, David H Jeffrey, Peter L Biochemistry 9:1423-8 3049072 Pubmed 1988 Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex Hoefsloot, Lies H Hoogeveen-Westerveld, Marianne Kroos, Marian A van Beeumen, Jos Reuser, Arnold J J Oostra, Ben A EMBO J. 7:1697-704 Reactome Database ID Release 81 10306371 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10306371 Reactome R-SSC-70221 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70221.1 GENE ONTOLOGY GO:0005980 Cytosolic glycogen breakdown occurs via the same chemical steps in all tissues but is separately regulated via tissue specific isozymes and signaling pathways that enable distinct physiological fates for liver glycogen and that in other tissues. Glycogen phosphorylase, which can be activated by phosphorylase kinase, catalyzes the removal of glucose residues as glucose 1-phosphate from the ends of glycogen branches. The final four residues of each branch are removed in two steps catalyzed by debranching enzyme, and further glycogen phosphorylase activity completes the process of glycogen breakdown. The figure shows the actions of phosphorylase and debranching enzyme. The first glucose residue in each branch is released as free glucose; all other residues are released as glucose 1-phosphate. The latter molecule can be converted to glucose 6-phosphate in a step shared with other pathways (Villar-Palasi and Larner 1970; Hers 1976).<p>Glycogen can also be taken up into lysosomes, where it is normally broken done by the action of a single enzyme, lysosomal alpha-glucosidase (GAA).<p>Enzymes in liver generate 1,5-anhydro-D-fructose from glycogen, which in turn can be reduced to 1,5-anhydro-D-glucitol, a sequence of events that may represent a novel minor pathway for glycogen breakdown (Kametani et al. 1996). 9022716 Pubmed 1996 Hepatic production of 1,5-anhydrofructose and 1,5-anhydroglucitol in rat by the third glycogenolytic pathway Kametani, Shunichi Shiga, Yoko Akanuma, Hiroshi Eur J Biochem 242:832-8 4320262 Pubmed 1970 Glycogen metabolism and glycolytic enzymes Villar-Palasi, C Larner, J Annu Rev Biochem 39:639-72 183599 Pubmed 1976 The control of glycogen metabolism in the liver Hers, Henri-Gery Annu Rev Biochem 45:167-89 Reactome Database ID Release 81 10306293 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10306293 Reactome R-SSC-8982491 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-8982491.1 GENE ONTOLOGY GO:0005977 Glycogen, a highly branched glucose polymer, is formed and broken down in most human tissues, but is most abundant in liver and muscle, where it serves as a major stored fuel. Glycogen metabolism has been studied in most detail in liver and skeletal muscle. Glycogen metabolism in other tissues has not been studied as extensively, and is thought to resemble the muscle process.<br>Glycogen synthesis involves five reactions. The first two, conversion of glucose 6-phosphate to glucose 1-phosphate and synthesis of UDP-glucose from glucose 1-phosphate and UTP, are shared with several other pathways. The next three reactions, the auto-catalyzed synthesis of a glucose oligomer on glycogenin, the linear extension of the glucose oligomer catalyzed by glycogen synthase, and the formation of branches catalyzed by glycogen branching enzyme, are unique to glycogen synthesis. Repetition of the last two reactions generates large, extensively branched glycogen polymers. The catalysis of glycogenin glucosylation and oligoglucose chain extension by distinct isozymes in liver and nonhepatic tissues allows them to be regulated independently (Agius 2008; Bollen et al. 1998; Roach et al. 2012).<br>Cytosolic glycogen breakdown occurs via the same chemical steps in all tissues but is separately regulated via tissue specific isozymes and signaling pathways that enable distinct physiological fates for glycogen in liver and other tissues. Glycogen phosphorylase, which can be activated by phosphorylase kinase, catalyzes the removal of glucose residues as glucose 1-phosphate from the ends of glycogen branches. The final four residues of each branch are removed in two steps catalyzed by debranching enzyme, and further glycogen phosphorylase activity completes the process of glycogen breakdown. The first glucose residue in each branch is released as free glucose; all other residues are released as glucose 1-phosphate. The latter molecule can be converted to glucose 6-phosphate in a step shared with other pathways (Villar-Palasi & Larner 1970; Hers 1976).<br>Glycogen can also be taken up into lysosomes, where it is normally broken done by the action of a single enzyme, lysosomal alpha-glucosidase (GAA) (Brown et al. 1970). Glucose metabolism Glycolysis and gluconeogenesis 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> Gluconeogenesis 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-RIGHT 6.4.1.1 Pyruvate + CO2 + ATP => ADP + Orthophosphate + Oxaloacetate Pyruvate, CO2, and ATP react to form oxaloacetate, ADP, and orthophosphate 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> CO2 carbon dioxide Reactome DB_ID: 29376 mitochondrial matrix GENE ONTOLOGY GO:0005759 carbon dioxide [ChEBI:16526] carbon dioxide ChEBI CHEBI:16526 Reactome Database ID Release 81 29376 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29376 Reactome R-ALL-29376 3 Reactome stable identifier. Use this 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-29376.3 COMPOUND C00011 PYR Pyruvate pyruvic acid 2-Oxopropanoate 2-Oxopropanoic acid Pyroracemic acid Reactome DB_ID: 113557 pyruvate [ChEBI:15361] pyruvate 2-oxopropanoate 2-oxopropanoic acid, ion(1-) ChEBI CHEBI:15361 Reactome Database ID Release 81 113557 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113557 Reactome R-ALL-113557 4 Reactome stable identifier. Use this 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-113557.4 COMPOUND C00022 ATP Adenosine 5'-triphosphate ATP(4-) Reactome DB_ID: 113593 Reactome Database ID Release 81 113593 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113593 Reactome R-ALL-113593 4 Reactome stable identifier. Use this 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-113593.4 COMPOUND C00002 Pi Orthophosphate hydrogenphosphate Phosphate Reactome DB_ID: 113548 Reactome Database ID Release 81 113548 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113548 Reactome R-ALL-113548 4 Reactome stable identifier. Use this 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-113548.4 COMPOUND C00009 ADP Adenosine 5'-diphosphate ADP(3-) Reactome DB_ID: 113581 Reactome Database ID Release 81 113581 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113581 Reactome R-ALL-113581 4 Reactome stable identifier. Use this 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-113581.4 COMPOUND C00008 OAA Oxaloacetate oxaloacetic acid Oxalacetic acid 2-oxobutanedioic acid Oxosuccinic acid Reactome DB_ID: 113587 oxaloacetate(2-) [ChEBI:16452] oxaloacetate(2-) oxosuccinate oxaloacetate dianion oxobutanedioic acid, ion(2-) oxaloacetate oxobutanedioate ChEBI CHEBI:16452 Reactome Database ID Release 81 113587 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113587 Reactome R-ALL-113587 5 Reactome stable identifier. Use this 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-113587.5 COMPOUND C00036 ACTIVATION pyruvate carboxylase holoenzyme Reactome DB_ID: 10207557 Btn D-Biotin biotin Vitamin H Coenzyme R Reactome DB_ID: 29582 biotin [ChEBI:15956] biotin ChEBI CHEBI:15956 Reactome Database ID Release 81 29582 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29582 Reactome R-ALL-29582 3 Reactome stable identifier. Use this 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-29582.3 COMPOUND C00120 4 PC PC F1RUV5 Reactome DB_ID: 10207555 UniProt:F1RUV5 PC UniProt F1RUV5 21 EQUAL 1178 EQUAL Reactome Database ID Release 81 10207555 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207555 Reactome R-SSC-70498 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70498.1 4 Mn2+ Manganese manganese(2+) Mn(II) Reactome DB_ID: 113527 Reactome Database ID Release 81 113527 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113527 Reactome R-ALL-113527 3 Reactome stable identifier. Use this 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-113527.3 COMPOUND C00034 1 Reactome Database ID Release 81 10207557 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207557 Reactome R-SSC-70499 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70499.1 GENE ONTOLOGY GO:0004736 Reactome Database ID Release 81 10207558 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207558 Reactome Database ID Release 81 10207560 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207560 Reactome R-SSC-70501 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70501.1 The carboxylation of pyruvate to form oxaloacetate, catalyzed by mitochondrial pyruvate carboxylase, is an irreversible and allosterically regulated reaction (Jitrapakdee and Wallace 1999). 10229653 Pubmed 1999 Structure, function and regulation of pyruvate carboxylase Jitrapakdee, S Wallace, JC Biochem J 340:1-16 12437512 Pubmed 2003 Expression and characterization of a human pyruvate carboxylase variant by retroviral gene transfer Carbone, MA Robinson, BH Biochem J 370:275-282 ACTIVATION Reactome Database ID Release 81 71425 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=71425 Reactome R-HSA-71425 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-71425.1 Ac-CoA Acetyl coenzyme A acetyl-CoA Reactome DB_ID: 113559 acetyl-CoA(4-) [ChEBI:57288] acetyl-CoA(4-) acetyl-CoA 3'-phosphonatoadenosine 5'-(3-{(3R)-4-[(3-{[2-(acetylsulfanyl)ethyl]amino}-3-oxopropyl)amino]-3-hydroxy-2,2-dimethyl-4-oxobutyl} diphosphate) acetyl-coenzyme A(4-) acetyl-CoA tetraanion AcCoA(4-) ChEBI CHEBI:57288 Reactome Database ID Release 81 113559 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113559 Reactome R-ALL-113559 4 Reactome stable identifier. Use this 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-113559.4 COMPOUND C00024 LEFT-TO-RIGHT 1.1.1.37 Oxaloacetate + NADH + H+ <=> (S)-Malate + NAD+ 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> H+ hydron Reactome DB_ID: 113529 hydron [ChEBI:15378] hydron ChEBI CHEBI:15378 Reactome Database ID Release 81 113529 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113529 Reactome R-ALL-113529 4 Reactome stable identifier. Use this 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-113529.4 COMPOUND C00080 NADH Reactome DB_ID: 29362 NADH(2-) [ChEBI:57945] NADH(2-) NADH dianion adenosine 5'-{3-[1-(3-carbamoyl-1,4-dihydropyridin-1-yl)-1,4-anhydro-D-ribitol-5-yl] diphosphate} NADH ChEBI CHEBI:57945 Reactome Database ID Release 81 29362 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29362 Reactome R-ALL-29362 3 Reactome stable identifier. Use this 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-29362.3 COMPOUND C00004 MAL (S)-Malate (S)-malic acid L-Malate L-Apple acid L-Malic acid L-2-Hydroxybutanedioic acid Reactome DB_ID: 113544 (S)-malate(2-) [ChEBI:15589] (S)-malate(2-) (S)-malate ChEBI CHEBI:15589 Reactome Database ID Release 81 113544 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113544 Reactome R-ALL-113544 4 Reactome stable identifier. Use this 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-113544.4 COMPOUND C00149 NAD NAD+ NAD(+) Nicotinamide adenine dinucleotide DPN Diphosphopyridine nucleotide NAD+ Reactome DB_ID: 113526 NAD(1-) [ChEBI:57540] NAD(1-) adenosine 5'-{3-[1-(3-carbamoylpyridinio)-1,4-anhydro-D-ribitol-5-yl] diphosphate} NAD(+) NAD anion ChEBI CHEBI:57540 Reactome Database ID Release 81 113526 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113526 Reactome R-ALL-113526 5 Reactome stable identifier. Use this 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-113526.5 COMPOUND C00003 ACTIVATION MDH2 dimer Reactome DB_ID: 10208040 MDH2 MDH2 A0A5G2RGL7 Reactome DB_ID: 10208038 UniProt:A0A5G2RGL7 MDH2 UniProt A0A5G2RGL7 25 EQUAL 338 EQUAL Reactome Database ID Release 81 10208038 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208038 Reactome R-SSC-70977 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70977.1 2 Reactome Database ID Release 81 10208040 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208040 Reactome R-SSC-198511 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-198511.1 GENE ONTOLOGY GO:0030060 Reactome Database ID Release 81 10208041 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208041 Reactome Database ID Release 81 10208690 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208690 Reactome R-SSC-71783 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71783.1 Mitochondrial malate dehydrogenase catalyzes the reversible reaction of oxaloacetate and NADH + H+ to form malate and NAD+ (Luo et al. 2006). Unpublished crystallographic data indicate that the protein is a dimer (PDB 3E04). 16740313 Pubmed 2006 An NADH-tetrazolium-coupled sensitive assay for malate dehydrogenase in mitochondria and crude tissue homogenates Luo, C Wang, X Long, J Liu, J J Biochem Biophys Methods 68:101-11 9792106 Pubmed 1998 Aggregation states of mitochondrial malate dehydrogenase Sanchez, SA Hazlett, TL Brunet, JE Jameson, DM Protein Sci 7:2184-2189 LEFT-TO-RIGHT malate [mitochondrial matrix] + orthophosphate [cytosol] <=> malate [cytosol] + orthophosphate [mitochondrial matrix] 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> MAL (S)-Malate (S)-malic acid L-Malate L-Apple acid L-Malic acid L-2-Hydroxybutanedioic acid Reactome DB_ID: 198498 Reactome Database ID Release 81 198498 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=198498 Reactome R-ALL-198498 4 Reactome stable identifier. Use this 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-198498.4 COMPOUND C00149 ACTIVATION SLC25A10 SLC25A10 A0A287ASR0 Reactome DB_ID: 10229047 mitochondrial inner membrane GENE ONTOLOGY GO:0005743 UniProt:A0A287ASR0 SLC25A10 UniProt A0A287ASR0 1 EQUAL 287 EQUAL Reactome Database ID Release 81 10229047 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10229047 Reactome R-SSC-372848 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372848.1 GENE ONTOLOGY GO:0005310 Reactome Database ID Release 81 10229048 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10229048 Reactome Database ID Release 81 10229050 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10229050 Reactome R-SSC-372843 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372843.1 SLC25A10, the mitochondrial dicarboxylate carrier protein in the inner mitochondrial membrane, mediates the reversible exchange of mitochondrial malate for cytosolic phosphate (Fiermonte et al. 1999). 10585886 Pubmed 1999 Organization and sequence of the gene for the human mitochondrial dicarboxylate carrier: evolution of the carrier family Fiermonte, Giuseppe Dolce, V Arrigoni, R Runswick, MJ Walker, JE Palmieri, Ferdinando Biochem J 344:953-60 LEFT-TO-RIGHT 1.1.1.37 malate + NAD+ <=> oxaloacetate + NADH + H+ (S)-malate + NAD+ <=> oxaloacetate + NADH + H+ 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> NAD NAD+ NAD(+) Nicotinamide adenine dinucleotide DPN Diphosphopyridine nucleotide Reactome DB_ID: 29360 Reactome Database ID Release 81 29360 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29360 Reactome R-ALL-29360 5 Reactome stable identifier. Use this 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-29360.5 COMPOUND C00003 H+ proton hydron Reactome DB_ID: 70106 Reactome Database ID Release 81 70106 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=70106 Reactome R-ALL-70106 4 Reactome stable identifier. Use this 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-70106.4 COMPOUND C00080 OA Oxaloacetate oxaloacetic acid Oxalacetic acid 2-oxobutanedioic acid Oxosuccinic acid Reactome DB_ID: 76213 Reactome Database ID Release 81 76213 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=76213 Reactome R-ALL-76213 4 Reactome stable identifier. Use this 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-76213.4 COMPOUND C00036 NADH Reactome DB_ID: 73473 Reactome Database ID Release 81 73473 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=73473 Reactome R-ALL-73473 4 Reactome stable identifier. Use this 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-73473.4 COMPOUND C00004 ACTIVATION malate dehydrogenase 1 dimer Reactome DB_ID: 10223262 MDH1 MDH1 P11708 Reactome DB_ID: 10223260 UniProt:P11708 MDH1 UniProt P11708 2 EQUAL 334 EQUAL Reactome Database ID Release 81 10223260 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223260 Reactome R-SSC-198471 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-198471.1 2 Reactome Database ID Release 81 10223262 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223262 Reactome R-SSC-198437 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-198437.1 Reactome Database ID Release 81 10223263 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223263 Reactome Database ID Release 81 10223265 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223265 Reactome R-SSC-198508 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-198508.1 Cytosolic malate dehydrogenase catalyzes the reaction of malate and NAD+ to form oxaloacetate and NADH + H+ (Lo et al. 2005). The dimeric structure of the human dehydrogenase is inferred from that established for its well-studied pig homolog (Birktoft et al. 1989). 15565635 Pubmed 2005 Developmental regulation and cellular distribution of human cytosolic malate dehydrogenase (MDH1) Lo, AS-Y Liew, C-T Ngai, S-M Tsui, SK-W Fung, K-P Lee, C-Y Waye, MM-Y J Cell Biochem 94:763-773 2775751 Pubmed 1989 Refined crystal structure of cytoplasmic malate dehydrogenase at 2.5-A resolution Birktoft, JJ Rhodes, G Banaszak, LJ Biochemistry 28:6065-6081 LEFT-TO-RIGHT 2.6.1.1 oxaloacetate + glutamate <=> aspartate + alpha-ketoglutarate [GOT2] 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> Glu L-Glu L-glutamate(1-) L-glutamic acid L-Glutamate L-Glutaminic acid Reactome DB_ID: 113552 L-glutamate(1-) [ChEBI:29985] L-glutamate(1-) L-glutamic acid, ion(1-) [NH3+][C@@H](CCC([O-])=O)C([O-])=O WHUUTDBJXJRKMK-VKHMYHEASA-M C5H8NO4 146.12136 (2S)-2-ammoniopentanedioate hydrogen L-glutamate L-glutamate L-glutamic acid monoanion InChI=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/s1 ChEBI CHEBI:29985 Reactome Database ID Release 81 113552 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113552 Reactome R-ALL-113552 5 Reactome stable identifier. Use this 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-113552.5 COMPOUND C00025 2OG 2-Oxoglutarate 2-oxoglutaric acid Oxoglutaric acid 2-Ketoglutaric acid alpha-Ketoglutaric acid 2-oxoglutarate(2-) Reactome DB_ID: 113594 2-oxoglutarate(2-) [ChEBI:16810] 2-oxoglutarate(2-) 2-ketoglutarate alpha-ketoglutarate 2-oxopentanedioic acid, ion(2-) 2-oxoglutarate ChEBI CHEBI:16810 Reactome Database ID Release 81 113594 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113594 Reactome R-ALL-113594 4 Reactome stable identifier. Use this 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-113594.4 COMPOUND C00026 L-Asp L-Aspartate L-aspartate(1-) L-aspartic acid 2-aminosuccinic acid Reactome DB_ID: 113553 L-aspartate(1-) [ChEBI:29991] L-aspartate(1-) hydrogen L-aspartate C4H6NO4 L-aspartate (2S)-2-ammoniobutanedioate L-aspartic acid monoanion CKLJMWTZIZZHCS-REOHCLBHSA-M InChI=1S/C4H7NO4/c5-2(4(8)9)1-3(6)7/h2H,1,5H2,(H,6,7)(H,8,9)/p-1/t2-/m0/s1 132.09478 (2S)-2-ammoniosuccinate [NH3+][C@@H](CC([O-])=O)C([O-])=O ChEBI CHEBI:29991 Reactome Database ID Release 81 113553 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113553 Reactome R-ALL-113553 5 Reactome stable identifier. Use this 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-113553.5 COMPOUND C00049 ACTIVATION GOT2 dimer Reactome DB_ID: 10207699 GOT2 PXLP-K279-GOT2 P00506 Reactome DB_ID: 10207697 UniProt:P00506 GOT2 UniProt P00506 279 EQUAL 30 EQUAL 430 EQUAL Reactome Database ID Release 81 10207697 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207697 Reactome R-SSC-9608413 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-9608413.1 2 Reactome Database ID Release 81 10207699 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207699 Reactome R-SSC-70594 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70594.1 GENE ONTOLOGY GO:0004069 Reactome Database ID Release 81 10207700 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207700 Reactome Database ID Release 81 10207741 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207741 Reactome R-SSC-70613 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70613.1 Mitochondrial aspartate aminotransferase catalyzes the reversible reaction of oxaloacetate and glutamate to form aspartate and 2-oxoglutarate (alpha-ketoglutarate) (Martini et al. 1985). The active form of the enzyme is inferred to be a dimer with one molecule of pyridoxal phosphate associated with each monomer. 4052435 Pubmed 1985 The primary structure of mitochondrial aspartate aminotransferase from human heart Martini, F Angelaccio, S Barra, D Pascarella, S Maras, B Doonan, S Bossa, F Biochim Biophys Acta 832:46-51 LEFT-TO-RIGHT Exchange of malate and alpha-ketoglutarate (2-oxoglutarate) across the inner mitochondrial membrane malate [mitochondrial matrix] + alpha-ketoglutarate [cytosol] <=> malate [cytosol] + alpha-ketoglutarate [mitochondrial matrix] 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> 2OG 2-oxoglutarate 2-oxoglutaric acid Oxoglutaric acid 2-Ketoglutaric acid alpha-Ketoglutaric acid 2-Oxoglutarate alpha-ketoglutarate Reactome DB_ID: 29406 Reactome Database ID Release 81 29406 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29406 Reactome R-ALL-29406 4 Reactome stable identifier. Use this 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-29406.4 COMPOUND C00026 ACTIVATION SLC25A11 homodimer Reactome DB_ID: 10223253 F1RFX9 SLC25A11 SLC25A11 Reactome DB_ID: 10223251 UniProt:F1RFX9 SLC25A11 UniProt F1RFX9 2 EQUAL 314 EQUAL Reactome Database ID Release 81 10223251 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223251 Reactome R-SSC-198451 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-198451.1 2 Reactome Database ID Release 81 10223253 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223253 Reactome R-SSC-376856 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-376856.1 GENE ONTOLOGY GO:0015367 Reactome Database ID Release 81 10223254 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223254 Reactome Database ID Release 81 10223256 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223256 Reactome R-SSC-198440 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-198440.1 The SLC25A11 transport protein in the inner mitochondrial membrane mediates the reversible exchange of mitochondrial malate and cytosolic alpha-ketoglutarate (2-oxoglutarate). Qualitative evidence for this process comes from studies of the human protein (Kabe et al. 2006); kinetic details are inferred from stdies of rat mitochondria under conditions in which only the one transport protein appears to be active (Sluse et al. 1973). The dimeric state of the transport protein is inferred from studies of its bovine homologue (Bisaccia et al. 1996). 4687397 Pubmed 1973 Mechanism of the exchanges catalysed by the oxoglutarate translocator of rat-heart mitochondria. Kinetics of the external-product inhibition Sluse, FE Goffart, G Liébecq, C Eur J Biochem 32:283-91 8597574 Pubmed 1996 The formation of a disulfide cross-link between the two subunits demonstrates the dimeric structure of the mitochondrial oxoglutarate carrier Bisaccia, F Zara, V Capobianco, L Iacobazzi, V Mazzeo, M Palmieri, Ferdinando Biochim Biophys Acta 1292:281-88 16920706 Pubmed 2006 Porphyrin accumulation in mitochondria is mediated by 2-oxoglutarate carrier Kabe, Y Ohmori, M Sinouchi, K Tsuboi, Y Hirao, S Azuma, M Watanabe, H Okura, I Handa, Hiroshi J Biol Chem 281:31729–31735 LEFT-TO-RIGHT Exchange of alpha-ketoglutarate (2-oxoglutarate) and malate across the inner mitochondrial membrane malate [cytosol] + alpha-ketoglutarate [mitochondrial matrix] <=> malate [mitochondrial matrix] + alpha-ketoglutarate [cytosol] 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> ACTIVATION Reactome Database ID Release 81 10230133 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10230133 Reactome R-SSC-376851 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-376851.1 The SLC25A11 transport protein in the inner mitochondrial membrane mediates the reversible exchange of mitochondrial alpha-ketoglutarate (2-oxoglutarate) and cytosolic malate. Qualitative evidence for this process comes from studies of the human protein (Kabe et al. 2006); kinetic details are inferred from studies of rat mitochondria under conditions in which only the one transport protein appears to be active (Sluse et al. 1973). The dimeric state of the transport protein is inferred from studies of its bovine homologue (Bisaccia et al. 1996). LEFT-TO-RIGHT SLC25A12,13 exchange cytosolic L-Glu for mitochondrial matrix L-Asp aspartate [mitochondrial matrix] + glutamate [cytosol] => aspartate [cytosol] + glutamate [mitochondrial matrix] 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> L-Glu L-Glutamate L-glutamate(1-) L-glutamic acid L-Glutaminic acid Reactome DB_ID: 29404 Reactome Database ID Release 81 29404 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29404 Reactome R-ALL-29404 4 Reactome stable identifier. Use this 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-29404.4 COMPOUND C00025 L-Asp L-Aspartate L-aspartate(1-) L-aspartic acid 2-aminosuccinate Reactome DB_ID: 29448 Reactome Database ID Release 81 29448 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29448 Reactome R-ALL-29448 4 Reactome stable identifier. Use this 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-29448.4 COMPOUND C00049 ACTIVATION Converted from EntitySet in Reactome SLC25A12,13 mitochondrial aspartate glutamate carrier Reactome DB_ID: 10228977 SLC25A12 SLC25A12 A0A5G2QY62 Reactome DB_ID: 10228971 UniProt:A0A5G2QY62 SLC25A12 UniProt A0A5G2QY62 1 EQUAL 678 EQUAL Reactome Database ID Release 81 10228971 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10228971 Reactome R-SSC-372470 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372470.1 SLC25A13 SLC25A13 A0A5G2QD37 Reactome DB_ID: 10228975 UniProt:A0A5G2QD37 SLC25A13 UniProt A0A5G2QD37 1 EQUAL 675 EQUAL Reactome Database ID Release 81 10228975 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10228975 Reactome R-SSC-372485 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372485.1 Reactome Database ID Release 81 10228977 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10228977 Reactome R-SSC-372469 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372469.1 GENE ONTOLOGY GO:0015172 Reactome Database ID Release 81 10228978 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10228978 Reactome Database ID Release 81 10228980 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10228980 Reactome R-SSC-372448 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372448.1 Calcium-binding mitochondrial carrier proteins Aralar1 and Aralar2 (SLC25A12 and SLC25A13 respectively), located in the inner mitochondrial membrane, mediate the exchange of cytosolic aspartate and mitochondrial glutamate (Palmieri et al. 2001). The exchange is physiologically irreversible because of the potential across the inner mitochondrial membrane (positive outside, negative inside). In the body, SLC25A12 is found mainly in heart, skeletal muscle, and brain, while SCL25A13 is widely expressed but most abundant in liver (del Arco et al. 2000; Palmieri et al. 2001). Defects in SLC25A13 are associated with type II citrullinemia, characterised by a liver-specific deficiency of the urea cycle enzyme argininosuccinate synthase (Kobayashi et al. 1999, Saheki et al. 2002). 10369257 Pubmed 1999 The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein Kobayashi, K Sinasac, DS Iijima, M Boright, AP Begum, L Lee, JR Yasuda, T Ikeda, S Hirano, R Terazono, H Crackower, MA Kondo, I Tsui, LC Scherer, SW Saheki, T Nat Genet 22:159-63 10642534 Pubmed 2000 Characterization of a second member of the subfamily of calcium-binding mitochondrial carriers expressed in human non-excitable tissues del Arco, A Agudo, M Satrústegui, J Biochem J 345:725-32 11566871 Pubmed 2001 Citrin and aralar1 are Ca(2+)-stimulated aspartate/glutamate transporters in mitochondria Palmieri, L Pardo, B Lasorsa, F M del Arco, A Kobayashi, K Iijima, M Runswick, MJ Walker, JE Saheki, T Satrústegui, J Palmieri, Ferdinando EMBO J 20:5060-9 12602510 Pubmed 2002 Pathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency Saheki, T Kobayashi, K Iijima, M Nishi, Ikumi Yasuda, T Yamaguchi, Naoki Gao, Hong Zhi Jalil, Md Abdul Begum, L Li, Meng Xian Metab Brain Dis 17:335-46 LEFT-TO-RIGHT 2.6.1.1 PXLP-K259-GOT1 dimer deaminates L-Asp aspartate + alpha-ketoglutarate <=> oxaloacetate + glutamate [GOT1] 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> ACTIVATION PXLP-K259-GOT1 dimer Reactome DB_ID: 10207678 GOT1 PXLP-K259-GOT1 P00503 Reactome DB_ID: 10207676 UniProt:P00503 GOT1 UniProt P00503 259 EQUAL 2 EQUAL 413 EQUAL Reactome Database ID Release 81 10207676 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207676 Reactome R-SSC-1295497 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-1295497.1 2 Reactome Database ID Release 81 10207678 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207678 Reactome R-SSC-70579 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70579.1 Reactome Database ID Release 81 10207679 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207679 Reactome Database ID Release 81 10207692 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207692 Reactome R-SSC-70592 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70592.1 Cytosolic aspartate aminotransferase (glutamate oxaloacetate transaminase 1 - GOT1) catalyzes the reversible reaction of aspartate and 2-oxoglutarate (alpha-ketoglutarate) to form oxaloacetate and glutamate (Doyle et al. 1990). Unpublished crystallographic data (PBD 3IIO) suggest the enzyme is a homodimer). 2241899 Pubmed 1990 The amino acid sequence of cytosolic aspartate aminotransferase from human liver Doyle, JM Schinina, ME Bossa, F Doonan, S Biochem J 270:651-7 LEFT-TO-RIGHT 4.1.1.32 oxaloacetate + GTP => phosphoenolpyruvate + GDP + CO2 [cytosol] Oxaloacetate and GTP react to form phosphoenolpyruvate, CO2, and GDP [cytosol] 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> GTP Guanosine 5'-triphosphate GTP)(4-) Reactome DB_ID: 29438 GTP(4-) [ChEBI:37565] GTP(4-) GTP gtp guanosine 5'-triphosphate(4-) ChEBI CHEBI:37565 Reactome Database ID Release 81 29438 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29438 Reactome R-ALL-29438 4 Reactome stable identifier. Use this 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-29438.4 COMPOUND C00044 PEP phosphoenolpyruvate Phosphoenolpyruvic acid Reactome DB_ID: 29492 phosphonatoenolpyruvate [ChEBI:58702] phosphonatoenolpyruvate phosphoenolpyruvate ChEBI CHEBI:58702 Reactome Database ID Release 81 29492 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29492 Reactome R-ALL-29492 4 Reactome stable identifier. Use this 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-29492.4 CO2 carbon dioxide Reactome DB_ID: 113528 Reactome Database ID Release 81 113528 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113528 Reactome R-ALL-113528 3 Reactome stable identifier. Use this 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-113528.3 COMPOUND C00011 GDP Guanosine 5'-diphosphate Guanosine diphosphate GDP(3-) Reactome DB_ID: 29420 GDP(3-) [ChEBI:58189] GDP(3-) 5'-O-[(phosphonatooxy)phosphinato]guanosine guanosine 5'-diphosphate(3-) GDP guanosine 5'-diphosphate trianion guanosine 5'-diphosphate GDP trianion ChEBI CHEBI:58189 Reactome Database ID Release 81 29420 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29420 Reactome R-ALL-29420 3 Reactome stable identifier. Use this 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-29420.3 COMPOUND C00035 ACTIVATION PCK1 PCK1 A0A4X1UM84 Reactome DB_ID: 10207316 UniProt:A0A4X1UM84 PCK1 UniProt A0A4X1UM84 1 EQUAL 622 EQUAL Reactome Database ID Release 81 10207316 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207316 Reactome R-SSC-70495 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70495.1 GENE ONTOLOGY GO:0004613 Reactome Database ID Release 81 10207317 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207317 Reactome Database ID Release 81 10207319 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207319 Reactome R-SSC-70241 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70241.1 The transfer of a high-energy phosphate bond from GTP to oxaloacetate, to form phosphoenolpyruvate, GDP, and CO2, is catalyzed by cytosolic phosphoenolpyruvate carboxykinase (Dunten et al. 2002). This reaction is irreversible under physiological conditions. 11851336 Pubmed 2002 Crystal structure of human cytosolic phosphoenolpyruvate carboxykinase reveals a new GTP-binding site Dunten, P Belunis, C Crowther, R Hollfelder, K Kammlott, U Levin, W Michel, H Ramsey, GB Swain, A Weber, D Wertheimer, SJ J Mol Biol 316:257-264 LEFT-TO-RIGHT 4.1.1.32 oxaloacetate + GTP => phosphoenolpyruvate + GDP + CO2 [mitochondrial matrix] 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> GTP Guanosine 5'-triphosphate GTP)(4-) Reactome DB_ID: 113573 Reactome Database ID Release 81 113573 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113573 Reactome R-ALL-113573 3 Reactome stable identifier. Use this 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-113573.3 COMPOUND C00044 GDP Guanosine 5'-diphosphate Guanosine diphosphate GDP(3-) Reactome DB_ID: 113525 Reactome Database ID Release 81 113525 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113525 Reactome R-ALL-113525 3 Reactome stable identifier. Use this 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-113525.3 COMPOUND C00035 PEP phosphoenolpyruvate Phosphoenolpyruvic acid Reactome DB_ID: 372364 Reactome Database ID Release 81 372364 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=372364 Reactome R-ALL-372364 4 Reactome stable identifier. Use this 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-372364.4 ACTIVATION PCK2 PCK2 A0A287AES5 Reactome DB_ID: 10229040 UniProt:A0A287AES5 PCK2 UniProt A0A287AES5 33 EQUAL 640 EQUAL Reactome Database ID Release 81 10229040 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10229040 Reactome R-SSC-372815 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372815.1 Reactome Database ID Release 81 10229041 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10229041 Reactome Database ID Release 81 10229043 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10229043 Reactome R-SSC-372819 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372819.1 PCK2 (phosphoenolcarboxykinase), located in the mitochondrial matrix, catalyzes the physiologically irreversible reaction of oxaloacetate and GTP to form phosphoenolpyruvate, GDP, and CO2 (Modaressi et al. 1996, 1998). 9657976 Pubmed 1998 Human mitochondrial phosphoenolpyruvate carboxykinase 2 gene. Structure, chromosomal localization and tissue-specific expression Modaressi, S Brechtel, K Christ, B Jungermann, K Biochem J 333:359-66 8645161 Pubmed 1996 Molecular cloning, sequencing and expression of the cDNA of the mitochondrial form of phosphoenolpyruvate carboxykinase from human liver Modaressi, S Christ, B Bratke, J Zahn, S Heise, T Jungermann, K Biochem J 315:807-14 LEFT-TO-RIGHT Exchange of cytosolic citrate for mitochondrial phosphoenolpyruvate phosphoenolpyruvate [mitochondrial matrix] + citrate [cytosol] => phosphoenolpyruvate [cytosol] + citrate [mitochondrial matrix] 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> CIT Citrate citric acid Reactome DB_ID: 76190 citric acid [ChEBI:30769] citric acid ChEBI CHEBI:30769 Reactome Database ID Release 81 76190 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=76190 Reactome R-ALL-76190 3 Reactome stable identifier. Use this 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-76190.3 COMPOUND C00158 CIT Citrate citric acid 2-Hydroxy-1,2,3-propanetricarboxylic acid 2-Hydroxytricarballylic acid Reactome DB_ID: 29654 Reactome Database ID Release 81 29654 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29654 Reactome R-ALL-29654 3 Reactome stable identifier. Use this 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-29654.3 COMPOUND C00158 ACTIVATION SLC25A1 SLC25A1 A0A5G2QHR5 Reactome DB_ID: 10211979 UniProt:A0A5G2QHR5 SLC25A1 UniProt A0A5G2QHR5 14 EQUAL 311 EQUAL Reactome Database ID Release 81 10211979 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10211979 Reactome R-SSC-372445 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372445.1 GENE ONTOLOGY GO:0015142 Reactome Database ID Release 81 10211980 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10211980 Reactome Database ID Release 81 10228982 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10228982 Reactome R-SSC-372449 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372449.1 SLC25A1, in the inner mitochondrial membrane, mediates the exchange of cytosolic citrate for mitochondrial phosphoenolpyruvate. The exchange is physiologically irreversible because of the potential of the inner mitochondrial membrane. The gene encoding human SLC25A1 has been cloned and its expression pattern has been characterized (Heisterkamp et al. 1995; Iacobazzi et al. 1997), but the biochemical details of the transport process are inferred from those worked out for the well-characterized rat system (Robinson 1971; Soling et al. 1971; Kleineke et al. 1973). 8666394 Pubmed 1995 Localization of the human mitochondrial citrate transporter protein gene to chromosome 22Q11 in the DiGeorge syndrome critical region Heisterkamp, N Mulder, MP Langeveld, A ten Hoeve, J Wang, Z Roe, BA Groffen, J Genomics 29:451-6 9254007 Pubmed 1997 Organization and sequence of the human gene for the mitochondrial citrate transport protein Iacobazzi, V Lauria, G Palmieri, Ferdinando DNA Seq 7:127-39 11946196 Pubmed 1971 Effects of synthetic analogues of phosphoenolpyruvate on muscle and liver pyruvate kinase, muscle enolase, liver phosphoenolpyruvate carboxykinase and on the intra-/extra-mitochondrial tricarboxylic acid carrier transport system Söling, HD Walter, U Sauer, H Kleineke, J FEBS Lett 19:139-143 4719206 Pubmed 1973 On the specificity of the tricarboxylate carrier system in rat liver mitochondria Kleineke, J Sauer, H Söling, HD FEBS Lett 29:82-6 11945784 Pubmed 1971 Transport of phosphoenolpyruvate by the tricarboxylate transporting system in mammalian mitochondria Robinson, BH FEBS Lett 14:309-312 LEFT-TO-RIGHT 4.2.1.11 Phosphoenolpyruvate + H2O <=> 2-Phospho-D-glycerate 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> 2PG 2-Phospho-D-glycerate 2-phospho-D-glyceric acid D-Glycerate 2-phosphate Reactome DB_ID: 30485 2-phosphonato-D-glycerate(3-) [ChEBI:58289] 2-phosphonato-D-glycerate(3-) 2-phosphonato-D-glycerate (2R)-2-phosphoglycerate (2R)-3-hydroxy-2-(phosphonatooxy)propanoate 2-phosphonato-D-glycerate trianion ChEBI CHEBI:58289 Reactome Database ID Release 81 30485 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=30485 Reactome R-ALL-30485 4 Reactome stable identifier. Use this 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-30485.4 ACTIVATION Converted from EntitySet in Reactome enolase dimer Reactome DB_ID: 10207548 enolase 1 dimer, (alpha) Reactome DB_ID: 10207534 1 ENO1 ENO1 A0A287B6S5 Reactome DB_ID: 10207532 UniProt:A0A287B6S5 ENO1 UniProt A0A287B6S5 2 EQUAL 434 EQUAL Reactome Database ID Release 81 10207532 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207532 Reactome R-SSC-71437 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71437.1 2 Reactome Database ID Release 81 10207534 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207534 Reactome R-SSC-71438 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71438.1 enolase 2 dimer, (gamma, neuronal) Reactome DB_ID: 10207540 1 ENO2 ENO2 I3LCN1 Reactome DB_ID: 10207538 UniProt:I3LCN1 ENO2 UniProt I3LCN1 1 EQUAL 434 EQUAL Reactome Database ID Release 81 10207538 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207538 Reactome R-SSC-71429 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71429.1 2 Reactome Database ID Release 81 10207540 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207540 Reactome R-SSC-71430 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71430.1 enolase 3 dimer, (beta, muscle) Reactome DB_ID: 10207546 1 ENO3 ENO3 A0A286ZSQ1 Reactome DB_ID: 10207544 UniProt:A0A286ZSQ1 ENO3 UniProt A0A286ZSQ1 2 EQUAL 434 EQUAL Reactome Database ID Release 81 10207544 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207544 Reactome R-SSC-71433 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71433.1 2 Reactome Database ID Release 81 10207546 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207546 Reactome R-SSC-71434 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71434.1 Reactome Database ID Release 81 10207548 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207548 Reactome R-SSC-179497 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-179497.1 GENE ONTOLOGY GO:0004634 Reactome Database ID Release 81 10207549 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207549 Reactome Database ID Release 81 10207551 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207551 Reactome R-SSC-70494 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70494.1 Cytosolic enolase dimer catalyzes the reversible reaction of phosphoenolpyruvate and water to form 2-phosphoglycerate. Three enolase isozymes have been purified and biochemically characterized. The alpha isoform is widely expressed (Giallongo et al. 1986). The beta isoform is expressed in muscle. Evidence for its function in vivo in humans comes from studies of a patient in whom a point mutation in the gene encoding the enzyme was associated specifically with reduced enolase activity in muscle extracts, and with other symptoms consistent with a defect in glycolysis (Comi et al. 2001). The gamma isoform of human enolase is normally expressed in neural tissue. It is not known to have distinctive biochemical functions, but is of possible clinical interest as a marker of some types of neuroendocrine and lung tumors (McAleese et al. 1988). Verma and Kurl (1993) identifed a possible fourth isoform, a "lung-specific" enolase whose expression is increased in response to dexamethasone treatment. The protein has not been biochemically characterized, however, nor have the levels of mRNA and protein in other tissues been examined. Thus, the observation that this protein is particularly similar in its predicted amino acid sequence to a duck crystallin (Wistow et al. 1988) raises the possibility that its normal function is unrelated to glycolysis. 11506403 Pubmed 2001 Beta-enolase deficiency, a new metabolic myopathy of distal glycolysis Comi, GP Fortunato, F Lucchiari, S Bordoni, A Prelle, A Jann, S Keller, A Ciscato, P Galbiati, S Chiveri, L Torrente, Y Scarlato, G Bresolin, N Ann Neurol 50:202-7 3529090 Pubmed 1986 Molecular cloning and nucleotide sequence of a full-length cDNA for human alpha enolase Giallongo, A Feo, S Moore, R Croce, CM Showe, LC Proc Natl Acad Sci U S A 83:6741-5 3208766 Pubmed 1988 Complete amino acid sequence of the neurone-specific gamma isozyme of enolase (NSE) from human brain and comparison with the non-neuronal alpha form (NNE) McAleese, SM Dunbar, B Fothergill, JE Hinks, LJ Day, IN Eur J Biochem 178:413-7 7689884 Pubmed 1993 Human lung enolase: cloning and sequencing of cDNA and its inducibility with dexamethasone Verma, M Kurl, RN Biochem Mol Biol Int 30:293-303 2462567 Pubmed 1988 Tau-crystallin/alpha-enolase: one gene encodes both an enzyme and a lens structural protein Wistow, GJ Lietman, T Williams, LA Stapel, SO de Jong, WW Horwitz, J Piatigorsky, J J Cell Biol 107:2729-36 LEFT-TO-RIGHT 5.4.2.1 2-Phospho-D-glycerate <=> 3-Phospho-D-glycerate 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> 3PG 3-Phospho-D-glycerate 3-phospho-D-glyceric acid D-Glycerate 3-phosphate Reactome DB_ID: 29728 3-phosphonato-D-glycerate(3-) [ChEBI:58272] 3-phosphonato-D-glycerate(3-) (2R)-3-phosphoglycerate 3-phosphonato-D-glycerate 3-phosphonato-D-glycerate trianion (2R)-2-hydroxy-3-(phosphonatooxy)propanoate ChEBI CHEBI:58272 Reactome Database ID Release 81 29728 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29728 Reactome R-ALL-29728 4 Reactome stable identifier. Use this 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-29728.4 ACTIVATION Converted from EntitySet in Reactome PGAM dimers phosphoglycerate mutase dimer Reactome DB_ID: 10208463 phosphoglycerate mutase 1 dimer Reactome DB_ID: 10208455 PGAM1 PGAM1 A0A287AJQ2 Reactome DB_ID: 10208453 UniProt:A0A287AJQ2 PGAM1 UniProt A0A287AJQ2 2 EQUAL 254 EQUAL Reactome Database ID Release 81 10208453 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208453 Reactome R-SSC-71442 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71442.1 2 Reactome Database ID Release 81 10208455 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208455 Reactome R-SSC-71443 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71443.1 phosphoglycerate mutase 2 dimer Reactome DB_ID: 10208461 PGAM2 PGAM2 B5KJG2 Reactome DB_ID: 10208459 UniProt:B5KJG2 PGAM2 UniProt B5KJG2 1 EQUAL 253 EQUAL Reactome Database ID Release 81 10208459 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208459 Reactome R-SSC-70487 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70487.1 2 Reactome Database ID Release 81 10208461 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208461 Reactome R-SSC-70488 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70488.1 Reactome Database ID Release 81 10208463 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208463 Reactome R-SSC-179494 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-179494.1 GENE ONTOLOGY GO:0004619 Reactome Database ID Release 81 10208464 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208464 Reactome Database ID Release 81 10208466 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208466 Reactome R-SSC-71445 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71445.1 Cytosolic phosphoglycerate mutase dimer catalyzes the reversible isomerisation of 2- and 3-phosphoglycerate. There are two isoforms of this enzyme, PGAM1 (isoform B) and PGAM2 (isoform M). In the body, erythrocytes express only PGAM1, while skeletal muscle expresses only PGAM2. Other tissues express both isoforms (Repiso et al. 2005; Tsujino et al. 1993). 15710582 Pubmed 2005 Phosphoglycerate mutase BB isoenzyme deficiency in a patient with non-spherocytic anemia: familial and metabolic studies Repiso, A Baso, MJR Corrons, J-LV Carreras, J Climent, F Haematologica 90:257-259 8447317 Pubmed 1993 The molecular genetic basis of muscle phosphoglycerate mutase (PGAM) deficiency Tsujino, S Shanske, S Sakoda, S Fenichel, G DiMauro, S Am J Hum Genet 52:472-477 LEFT-TO-RIGHT 2.7.2.3 ATP + 3-Phospho-D-glycerate <=> ADP + 1,3-bisphospho-D-glycerate 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> 1,3BPG 3-phosphonato-D-glyceroyl phosphate(4-) 1,3-Bisphospho-D-glycerate 3-phospho-D-glyceroyl dihydrogen phosphate 3-Phospho-D-glyceroyl phosphate (R)-2-Hydroxy-3-(phosphonooxy)-1-monoanhydridewith phosphoric propanoic acid Reactome DB_ID: 29800 3-phosphonato-D-glyceroyl phosphate(4-) [ChEBI:57604] 3-phosphonato-D-glyceroyl phosphate(4-) 3-phosphonato-D-glyceroyl phosphate tetraanion (2R)-2-hydroxy-1-oxopropane-1,3-diyl bis(phosphate) (2R)-3-phospho-glyceroyl phosphate ChEBI CHEBI:57604 Reactome Database ID Release 81 29800 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29800 Reactome R-ALL-29800 5 Reactome stable identifier. Use this 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-29800.5 ACTIVATION Converted from EntitySet in Reactome PGK complexes Reactome DB_ID: 10207525 PGK1 complex Reactome DB_ID: 10207517 PGK1 PGK1 F1RPH0 Reactome DB_ID: 10207515 UniProt:F1RPH0 PGK1 UniProt F1RPH0 2 EQUAL 417 EQUAL Reactome Database ID Release 81 10207515 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207515 Reactome R-SSC-70483 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70483.1 1 1 Reactome Database ID Release 81 10207517 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207517 Reactome R-SSC-70484 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70484.1 PGK2 complex Reactome DB_ID: 10207523 1 PGK2 PGK2 Q6RI85 Reactome DB_ID: 10207521 UniProt:Q6RI85 PGK2 UniProt Q6RI85 1 EQUAL 417 EQUAL Reactome Database ID Release 81 10207521 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207521 Reactome R-SSC-6807622 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-6807622.1 1 Reactome Database ID Release 81 10207523 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207523 Reactome R-SSC-6807625 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-6807625.1 Reactome Database ID Release 81 10207525 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207525 Reactome R-SSC-6807624 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-6807624.1 GENE ONTOLOGY GO:0004618 Reactome Database ID Release 81 10207526 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207526 Reactome Database ID Release 81 10207528 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207528 Reactome R-SSC-70486 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70486.1 Cytosolic phosphoglycerate kinase complexed with magnesium catalyzes the reversible phosphorylation of 3-phosphoglycerate to form 1,3-bisphosphoglycerate (Huang et al. 1980a, 1980b). 6771269 Pubmed 1980 Complete amino acid sequence of human phosphoglycerate kinase. Isolation and amino acid sequence of tryptic peptides. Huang, IY Rubinfien, E Yoshida, A J Biol Chem 255:6408-11 7391027 Pubmed 1980 Complete amino acid sequence of human phosphoglycerate kinase. Cyanogen bromide peptides and complete amino acid sequence. Huang, IY Welch, CD Yoshida, A J Biol Chem 255:6412-20 LEFT-TO-RIGHT 1.2.1.12 1,3-bisphospho-D-glycerate + NADH + H+ <=> D-glyceraldehyde 3-phosphate + Orthophosphate + NAD+ 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> GA3P (2R)-2-Hydroxy-3-(phosphonooxy)-propanal D-glyceraldehyde 3-phosphate D-glyceraldehyde 3-phosphate(2-) Reactome DB_ID: 29578 D-glyceraldehyde 3-phosphate(2-) [ChEBI:59776] D-glyceraldehyde 3-phosphate(2-) D-glyceraldehyde 3-phosphate (2R)-2-hydroxy-3-oxopropyl phosphate D-glyceraldehyde 3-phosphate dianion ChEBI CHEBI:59776 Reactome Database ID Release 81 29578 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29578 Reactome R-ALL-29578 4 Reactome stable identifier. Use this 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-29578.4 COMPOUND C00118 ACTIVATION Converted from EntitySet in Reactome GAPDH tetramers glyceraldehyde-3-phosphate dehydrogenase tetramer Reactome DB_ID: 10207432 glyceraldehyde-3-phosphate dehydrogenase tetramer Reactome DB_ID: 10207424 GAPDH GAPDH P00355 Reactome DB_ID: 10207422 UniProt:P00355 GAPDH UniProt P00355 2 EQUAL 335 EQUAL Reactome Database ID Release 81 10207422 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207422 Reactome R-SSC-70428 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70428.1 4 Reactome Database ID Release 81 10207424 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207424 Reactome R-SSC-70447 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70447.1 glyceraldehyde-3-phosphate dehydrogenase, testis-specific, tetramer Reactome DB_ID: 10207430 GAPDHS GAPDHS F1RM74 Reactome DB_ID: 10207428 UniProt:F1RM74 GAPDHS UniProt F1RM74 1 EQUAL 408 EQUAL Reactome Database ID Release 81 10207428 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207428 Reactome R-SSC-372873 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372873.1 4 Reactome Database ID Release 81 10207430 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207430 Reactome R-SSC-372869 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372869.1 Reactome Database ID Release 81 10207432 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207432 Reactome R-SSC-372871 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372871.1 GENE ONTOLOGY GO:0004365 Reactome Database ID Release 81 10207433 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207433 Reactome Database ID Release 81 10207511 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207511 Reactome R-SSC-70482 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70482.1 The reversible reduction of 1,3-bisphosphoglycerate to form glyceraldehyde-3-phosphate is catalyzed by cytosolic glyceraldehyde-3-phosphate dehydrogenase tetramer.<p>There are multiple human glyceraldehyde 3-phosphate dehydrogenase-like pseudogenes, but only one glyceraldehyde 3-phosphate dehydrogenase gene expressed in somatic tissue (Benham and Povey 1989). Consistent with this conclusion, the homogeneous enzymes purified from various human tissues had indistinguishable physical and immunochemical properties (Heinz and Freimuller 1982), and studies of human erythrocytes of various ages suggested that variant forms of the enzyme arise as a result of post-translational modifications (Edwards et al. 1976). There is, however, an authentic second isoform of glyceraldehyde 3-phosphate dehydrogenase whose expression is confined to spermatogenic cells of the testis (Welch et al. 2000). 10714828 Pubmed 2000 Human glyceraldehyde 3-phosphate dehydrogenase-2 gene is expressed specifically in spermatogenic cells. Welch, JE Brown, PL O'Brien, DA Magyar, PL Bunch, DO Mori, C Eddy, EM J Androl 21:328-38 3170585 Pubmed 1988 Isolation and complete sequence of a functional human glyceraldehyde-3-phosphate dehydrogenase gene Ercolani, L Florence, B Denaro, M Alexander, M J Biol Chem 263:15335-41 7144574 Pubmed 1983 Glyceraldehyde-3-phosphate dehydrogenase from human tissues. Heinz, F Freimüller, B Methods Enzymol 89:301-5 183598 Pubmed 1976 Isozymes of glyceraldehyde-3-phosphate dehydrogenase in man and other mammals. Edwards, YH Clark, P Harris, H Ann Hum Genet 40:67-77 LEFT-TO-RIGHT 5.3.1.1 D-glyceraldehyde 3-phosphate <=> dihydroxyacetone phosphate 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> DHAP glycerone phosphate dihydroxyacetone phosphate glycerone phosphate(2-) Reactome DB_ID: 75970 glycerone phosphate(2-) [ChEBI:57642] glycerone phosphate(2-) dihydroxyacetone phosphate dianion glycerone phosphate dianion dihydroxyacetone phosphate 3-hydroxy-2-oxopropyl phosphate ChEBI CHEBI:57642 Reactome Database ID Release 81 75970 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=75970 Reactome R-ALL-75970 4 Reactome stable identifier. Use this 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-75970.4 COMPOUND C00111 ACTIVATION TPI1 dimer Reactome DB_ID: 10207441 TPI1 TPI1 A0A288CFT0 Reactome DB_ID: 10207439 UniProt:A0A288CFT0 TPI1 UniProt A0A288CFT0 1 EQUAL 286 EQUAL Reactome Database ID Release 81 10207439 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207439 Reactome R-SSC-70451 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70451.1 2 Reactome Database ID Release 81 10207441 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207441 Reactome R-SSC-70452 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70452.1 GENE ONTOLOGY GO:0004807 Reactome Database ID Release 81 10207442 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207442 Reactome Database ID Release 81 10207509 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207509 Reactome R-SSC-70481 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70481.1 The reversible conversion of glyceraldehyde-3-phosphate to dihydroxyacetone phosphate is catalyzed by cytosolic triose phosphate isomerase (Watanabe et al. 1996; Lu et al. 1984). 6434534 Pubmed 1984 Primary structure of human triosephosphate isomerase Lu, HS Yuan, PM Gracy, RW J Biol Chem 259:11958-68 8571957 Pubmed 1996 Molecular analysis of a series of alleles in humans with reduced activity at the triosephosphate isomerase locus Watanabe, M Zingg, BC Mohrenweiser, HW Am J Hum Genet 58:308-16 LEFT-TO-RIGHT 4.1.2.13 dihydroxyacetone phosphate + D-glyceraldehyde 3-phosphate <=> D-fructose 1,6-bisphosphate 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> F1,6PP D-Fructose 1,6-bisphosphate keto-D-fructose 1,6-bisphosphate beta-D-fructofuranose 1,6-bisphosphate(4-) Reactome DB_ID: 30014 beta-D-fructofuranose 1,6-bisphosphate(4-) [ChEBI:32966] beta-D-fructofuranose 1,6-bisphosphate(4-) beta-D-fructofuranose 1,6-bisphosphate beta-D-fructose 1,6-bisphosphate 1,6-di-O-phosphonato-beta-D-fructofuranose ChEBI CHEBI:32966 Reactome Database ID Release 81 30014 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=30014 Reactome R-ALL-30014 5 Reactome stable identifier. Use this 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-30014.5 COMPOUND C00354 ACTIVATION Converted from EntitySet in Reactome aldolase tetramer fructose bisphosphate aldolase tetramer Reactome DB_ID: 10208480 fructose-bisphosphate aldolase A, class I holoenzyme Reactome DB_ID: 10208472 ALDOA ALDOA A0A287BFY0 Reactome DB_ID: 10208470 UniProt:A0A287BFY0 ALDOA UniProt A0A287BFY0 2 EQUAL 364 EQUAL Reactome Database ID Release 81 10208470 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208470 Reactome R-SSC-71492 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71492.1 4 Reactome Database ID Release 81 10208472 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208472 Reactome R-SSC-71493 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71493.1 ALDOB tetramer Reactome DB_ID: 10207353 ALDOB ALDOB A0A5G2RHD6 Reactome DB_ID: 10207351 UniProt:A0A5G2RHD6 ALDOB UniProt A0A5G2RHD6 2 EQUAL 364 EQUAL Reactome Database ID Release 81 10207351 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207351 Reactome R-SSC-70339 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70339.1 4 Reactome Database ID Release 81 10207353 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207353 Reactome R-SSC-70340 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70340.1 fructose-bisphosphate aldolase C, class I holoenzyme Reactome DB_ID: 10208478 ALDOC ALDOC A0A287B8Z2 Reactome DB_ID: 10208476 UniProt:A0A287B8Z2 ALDOC UniProt A0A287B8Z2 2 EQUAL 364 EQUAL Reactome Database ID Release 81 10208476 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208476 Reactome R-SSC-70455 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70455.1 4 Reactome Database ID Release 81 10208478 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208478 Reactome R-SSC-70456 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70456.1 Reactome Database ID Release 81 10208480 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208480 Reactome R-SSC-179508 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-179508.1 GENE ONTOLOGY GO:0004332 Reactome Database ID Release 81 10208481 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208481 Reactome Database ID Release 81 10208483 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208483 Reactome R-SSC-71495 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71495.1 In this freely reversible cytosolic reaction, dihydroxyacetone phosphate and D-glyceraldehyde 3-phosphate react to form D-fructose 1,6-bisphosphate. The active form of aldolase, the enzyme that catalyzes the reaction, is a homotetramer. Three aldolase isozymes have been identified which differ in their patterns of expression in various adult tissues and during development but are otherwise functionally indistinguishable (Ali and Cox 1995; Dunbar and Fothergill-Gilmore 1988). 7717389 Pubmed 1995 Diverse mutations in the aldolase B gene that underlie the prevalence of hereditary fructose intolerance Ali, Manir Cox, Timothy M Am J Hum Genet 56:1002-5 3355497 Pubmed 1988 The complete amino acid sequence of human skeletal-muscle fructose-bisphosphate aldolase Dunbar, B Fothergill-Gilmore, LA Biochem J 249:779-88 LEFT-TO-RIGHT 3.1.3.11 D-fructose 1,6-bisphosphate + H2O => D-fructose 6-phosphate + orthophosphate 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> Fru(6)P D-Fructose 6-phosphate keto-D-fructose 6-phosphate D-Fructose 6-phosphoric acid Neuberg ester D-fructose 6-phosphate(2-) Reactome DB_ID: 29512 beta-D-fructofuranose 6-phosphate(2-) [ChEBI:57634] beta-D-fructofuranose 6-phosphate(2-) beta-D-fructofuranose 6-phosphate dianion 6-O-phosphonato-beta-D-fructofuranose ChEBI CHEBI:57634 Reactome Database ID Release 81 29512 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29512 Reactome R-ALL-29512 4 Reactome stable identifier. Use this 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-29512.4 COMPOUND C00085 ACTIVATION Converted from EntitySet in Reactome FBP tetramer fructose-1,6-bisphosphatase isoforms Reactome DB_ID: 10207504 FBP1 tetramer Reactome DB_ID: 10207496 4 FBP1 FBP1 P00636 Reactome DB_ID: 10207494 UniProt:P00636 FBP1 UniProt P00636 2 EQUAL 338 EQUAL Reactome Database ID Release 81 10207494 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207494 Reactome R-SSC-70476 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70476.1 4 Reactome Database ID Release 81 10207496 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207496 Reactome R-SSC-70477 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70477.1 FBP2 tetramer Reactome DB_ID: 10207502 4 FBP2 FBP2 A0A287AY74 Reactome DB_ID: 10207500 UniProt:A0A287AY74 FBP2 UniProt A0A287AY74 1 EQUAL 339 EQUAL Reactome Database ID Release 81 10207500 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207500 Reactome R-SSC-71500 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71500.1 4 Reactome Database ID Release 81 10207502 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207502 Reactome R-SSC-2267351 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-2267351.1 Reactome Database ID Release 81 10207504 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207504 Reactome R-SSC-372874 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-372874.1 GENE ONTOLOGY GO:0042132 Reactome Database ID Release 81 10207505 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207505 Reactome Database ID Release 81 10207507 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207507 Reactome R-SSC-70479 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70479.1 Cytosolic fructose-1,6-bisphosphatase catalyzes the physiologically irreversible hydrolysis of fructose-1,6-bisphosphate to form fructose-6-phosphate and orthophosphate. In the body, two isoforms of the enzyme are expressed, one ubiquitous and one muscle-specific (Kikawa et al. 1997; Tillmann and Eschrich 1998). 9382095 Pubmed 1997 Identification of genetic mutations in Japanese patients with fructose-1,6-bisphosphatase deficiency Kikawa, Y Inuzuka, M Jin, BY Kaji, S Koga, J-i Yamamoto, Y Fujisawa, K Hata, I Nakai, A Shigematsu, Y Mizunuma, H Taketo, A Mayumi, M Sudo, M Am J Hum Genet 61:852-861 9678974 Pubmed 1998 Isolation and characterization of an allelic cDNA for human muscle fructose-1,6-bisphosphatase Tillmann, H Eschrich, K Gene 212:295-304 INHIBITION Reactome Database ID Release 81 111539 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=111539 Reactome R-MMU-111539 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-MMU-111539.1 LEFT-TO-RIGHT 5.3.1.9 D-fructose 6-phosphate <=> alpha-D-Glucose 6-phosphate 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> ACTIVATION GPI dimer Reactome DB_ID: 10207485 GPI GPI P08059 Reactome DB_ID: 10207483 UniProt:P08059 GPI UniProt P08059 2 EQUAL 558 EQUAL Reactome Database ID Release 81 10207483 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207483 Reactome R-SSC-70468 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70468.1 2 Reactome Database ID Release 81 10207485 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207485 Reactome R-SSC-70469 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70469.1 GENE ONTOLOGY GO:0004347 Reactome Database ID Release 81 10207486 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207486 Reactome Database ID Release 81 10207490 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207490 Reactome R-SSC-70475 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70475.1 The reversible isomerization of fructose-6-phosphate to form glucose-6-phosphate is catalyzed by cytosolic phosphoglucose isomerase (Noltman 1972; Xu and Beutler 1994; Tsuboi et al. 1958). 13538944 Pubmed 1958 Enzymes of the human erythrocyte. IV. Phosphoglucose isomerase, purification and properties. Tsuboi, KK Estrada, J Hudson, PB J Biol Chem 231:19-29 7989588 Pubmed 1994 The characterization of gene mutations for human glucose phosphate isomerase deficiency associated with chronic hemolytic anemia Xu, W Beutler, Ernest J Clin Invest 94:2326-9 0121227022 ISBN 1972 Aldose-ketose isomerases Noltmann, EA The Enzymes, 3rd ed (Book): 271-354 LEFT-TO-RIGHT Cytosolic glucose 6-phosphate is exchanged for orthophosphate from the endoplasmic reticulum lumen by SLC37A4 alpha-D-glucose 6-phosphate (cytosol) + orthophosphate (ER lumen) => alpha-D-glucose 6-phosphate (ER lumen) + orthophosphate (cytosol) (SLC37A4) 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> Pi Orthophosphate hydrogenphosphate Phosphate Reactome DB_ID: 113551 endoplasmic reticulum lumen GENE ONTOLOGY GO:0005788 Reactome Database ID Release 81 113551 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113551 Reactome R-ALL-113551 4 Reactome stable identifier. Use this 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-113551.4 COMPOUND C00009 G6P alpha-D-glucose 6-phosphate(2-) alpha-D-glucose 6-phosphate Reactome DB_ID: 113782 Reactome Database ID Release 81 113782 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113782 Reactome R-ALL-113782 4 Reactome stable identifier. Use this 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-113782.4 COMPOUND C00668 ACTIVATION SLC37A4 SLC37A4 A0A287BS88 Reactome DB_ID: 10223269 endoplasmic reticulum membrane GENE ONTOLOGY GO:0005789 UniProt:A0A287BS88 SLC37A4 UniProt A0A287BS88 1 EQUAL 429 EQUAL Reactome Database ID Release 81 10223269 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223269 Reactome R-SSC-3006348 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3006348.1 GENE ONTOLOGY GO:0061513 Reactome Database ID Release 81 10223270 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223270 Reactome Database ID Release 81 10223272 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10223272 Reactome R-SSC-198513 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-198513.1 The SLC37A4 transport protein in the endoplasmic reticulum membrane mediates the exchange of cytosolic glucose-6-phosphate and orthophosphate from the endoplasmic reticulum lumen. Defects in this transporter are associated with glycogen storage disease type Ib (Gerin et al. 1997; Chen et al. 2008; Veiga-da-Cunha et al. 1998). 18337460 Pubmed 2008 The glucose-6-phosphate transporter is a phosphate-linked antiporter deficient in glycogen storage disease type Ib and Ic Chen, Shih-Yin Pan, Chi-Jiunn Nandigama, Krishnamachary Mansfield, Brian C Ambudkar, Suresh V Chou, Janice Y FASEB J. 22:2206-13 9428641 Pubmed 1997 Sequence of a putative glucose 6-phosphate translocase, mutated in glycogen storage disease type Ib Gerin, I Veiga-da-Cunha, M Achouri, Y Collet, JF Van Schaftingen, Emile FEBS Lett 419:235-238 9758626 Pubmed 1998 A gene on chromosome 11q23 coding for a putative glucose- 6-phosphate translocase is mutated in glycogen-storage disease types Ib and Ic Veiga-da-Cunha, M Gerin, I Chen, Yuan-Tsong de Barsy, Thierry de Lonlay, Pascale Dionisi-Vici, C Fenske, Christiane D Lee, Philip J Leonard, JV Maire, I McConkie-Rosell, A Schweitzer, Susanne Vikkula, Miikka Van Schaftingen, Emile Am. J. Hum. Genet. 63:976-83 LEFT-TO-RIGHT SLC37A1, SLC37A2 exchange G6P for Pi across the endoplasmic reticulum membrane alpha-D-glucose 6-phosphate (cytosol) + orthophosphate (ER lumen) => alpha-D-glucose 6-phosphate (ER lumen) + orthophosphate (cytosol) (SLC37A1 or SLC37A2) 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> ACTIVATION Converted from EntitySet in Reactome SLC37A1,2 Reactome DB_ID: 10265649 F1SG45 SLC37A1 SLC37A1 Reactome DB_ID: 10265643 UniProt:F1SG45 SLC37A1 UniProt F1SG45 1 EQUAL 533 EQUAL Reactome Database ID Release 81 10265643 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265643 Reactome R-SSC-3257123 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3257123.1 SLC37A2 SLC37A2-2 A0A287AX11 Reactome DB_ID: 10265647 UniProt:A0A287AX11 SLC37A2 UniProt A0A287AX11 1 EQUAL 505 EQUAL Reactome Database ID Release 81 10265647 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265647 Reactome R-SSC-3257124 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3257124.1 Reactome Database ID Release 81 10265649 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265649 Reactome R-SSC-3257118 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3257118.1 Reactome Database ID Release 81 10265650 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265650 Reactome Database ID Release 81 10265652 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265652 Reactome R-SSC-3257122 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3257122.1 GENE ONTOLOGY GO:0015760 The SLC37A1 and SLC37A2 transport proteins mediate the exchange of glucose-6-phosphate and orthophosphate across lipid bilayer membranes in vitro, and both proteins are located in the endoplasmic reticulum membrane. Their physiological function, however, is unknown (Pan et al. 2011; Chou et al. 2013). 21949678 Pubmed 2011 SLC37A1 and SLC37A2 are phosphate-linked, glucose-6-phosphate antiporters Pan, Chi-Jiunn Chen, Shih-Yin Jun, Hyun Sik Lin, Su Ru Mansfield, Brian C Chou, Janice Y PLoS ONE 6:e23157 23506893 Pubmed 2013 The SLC37 family of phosphate-linked sugar phosphate antiporters Chou, Janice Y Sik Jun, Hyun Mansfield, Brian C Mol. Aspects Med. 34:601-11 LEFT-TO-RIGHT 3.1.3.9 G6PC hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (liver) alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC - liver) 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> H2O water Reactome DB_ID: 113519 Reactome Database ID Release 81 113519 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113519 Reactome R-ALL-113519 3 Reactome stable identifier. Use this 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-113519.3 COMPOUND C00001 Glc alpha-D-glucose Reactome DB_ID: 113780 Reactome Database ID Release 81 113780 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113780 Reactome R-ALL-113780 3 Reactome stable identifier. Use this 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-113780.3 COMPOUND C00267 ACTIVATION G6PC G6PC1 D7EZN7 Reactome DB_ID: 10208721 UniProt:D7EZN7 G6PC1 UniProt D7EZN7 1 EQUAL 357 EQUAL Reactome Database ID Release 81 10208721 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208721 Reactome R-SSC-71823 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71823.1 GENE ONTOLOGY GO:0004346 Reactome Database ID Release 81 10208722 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208722 Reactome Database ID Release 81 10208724 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10208724 Reactome R-SSC-71825 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-71825.1 Glucose-6-phosphatase (G6PC) associated with the inner face of the endoplasmic reticulum membrane catalyzes the hydrolysis of glucose-6-phosphate to glucose and orthophosphate. This reaction is essentially irreversible (Lei et al. 1993, Ghosh et al. 2002). Defects in glucose-6-phosphatase are the cause of glycogen storage disease type 1a (Lei et al. 1993, 1995, Chou and Mansfield 2008). 7573034 Pubmed 1995 Genetic basis of glycogen storage disease type 1a: Prevalent mutations at the glucose-6-phosphatase locus Lei, K-J Chen, Yuan-Tsong Chen, H Wong, L-JC Liu, J-L McConkie-Rosell, A Van Hove, JLK Ou, HC-Y Yeh, NJ Pan, LY Chou, Janice Y Am J Hum Genet 57:766-771 8211187 Pubmed 1993 Mutations in the glucose-6-phosphatase gene that cause glycogen storage disease type 1a Lei, K-J Shelly, Leslie L Pan, Chi-Jiunn Sidbury, James B Chou, Janice Y Science 262:580-3 12093795 Pubmed 2002 The catalytic center of glucose-6-phosphatase. HIS176 is the nucleophile forming the phosphohistidine-enzyme intermediate during catalysis Ghosh, Abhijit Shieh, Jeng-Jer Pan, Chi-Jiunn Sun, Mao-Sen Chou, Janice Y J. Biol. Chem. 277:32837-42 18449899 Pubmed 2008 Mutations in the glucose-6-phosphatase-alpha (G6PC) gene that cause type Ia glycogen storage disease Chou, Janice Y Mansfield, Brian C Hum. Mutat. 29:921-30 11879177 Pubmed 2002 The glucose-6-phosphatase system Van Schaftingen, Emile Gerin, I Biochem J 362:513-532 LEFT-TO-RIGHT 3.1.3.9 G6PC2 hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (islet) alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC2 - islet) 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> ACTIVATION G6PC2 G6PC2 F1S1W4 Reactome DB_ID: 10265674 UniProt:F1S1W4 G6PC2 UniProt F1S1W4 1 EQUAL 355 EQUAL Reactome Database ID Release 81 10265674 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265674 Reactome R-SSC-981603 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-981603.1 Reactome Database ID Release 81 10265675 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265675 Reactome Database ID Release 81 10265677 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265677 Reactome R-SSC-3266566 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3266566.1 Glucose-6-phosphatase 2 (G6PC2), associated with the endoplasmic reticulum membrane catalyzes the hydrolysis of glucose-6-phosphate to glucose and orthophosphate. This reaction is essentially irreversible. This enzyme is expressed in islet cells of the pancreas (Petrolonis et al. 2004). 14722102 Pubmed 2004 Enzymatic characterization of the pancreatic islet-specific glucose-6-phosphatase-related protein (IGRP) Petrolonis, AJ Yang, Q Tummino, PJ Fish, SM Prack, AE Jain, S Parsons, TF Li, P Dales, NA Ge, L Langston, SP Schuller, AG An, WF Tartaglia, LA Chen, H Hong, SB J Biol Chem 279:13976-83 LEFT-TO-RIGHT 3.1.3.9 G6PC3 hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (ubiquitous) alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC3 - ubiquitous) 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> ACTIVATION G6PC3 G6PC3 F1S1J5 Reactome DB_ID: 10265656 UniProt:F1S1J5 G6PC3 UniProt F1S1J5 1 EQUAL 346 EQUAL Reactome Database ID Release 81 10265656 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265656 Reactome R-SSC-981605 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-981605.1 Reactome Database ID Release 81 10265657 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265657 Reactome Database ID Release 81 10265659 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10265659 Reactome R-SSC-3262512 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-3262512.1 Glucose-6-phosphatase 3 (G6PC3) associated with the endoplasmic reticulum membrane catalyzes the hydrolysis of glucose-6-phosphate to glucose and orthophosphate. This reaction is essentially irreversible (Guionie et al. 2003; Ghosh et al. 2004). In the body, this enzyme is ubiquitously expressed; mutations that inactivate it are associated with severe congenital neutropenia (but not with fasting hypoglycemia or lactic acidemia) (Boztug et al. 2009). 12965222 Pubmed 2003 Identification and characterisation of a new human glucose-6-phosphatase isoform Guionie, O Clottes, E Stafford, K Burchell, A FEBS Lett 551:159-64 14718531 Pubmed 2004 Histidine 167 is the phosphate acceptor in glucose-6-phosphatase-beta forming a phosphohistidine enzyme intermediate during catalysis Ghosh, Abhijit Shieh, Jeng-Jer Pan, Chi-Jiunn Chou, Janice Y J. Biol. Chem. 279:12479-83 19118303 Pubmed 2009 A syndrome with congenital neutropenia and mutations in G6PC3 Boztug, Kaan Appaswamy, Giridharan Ashikov, Angel Schäffer, Alejandro A Salzer, Ulrich Diestelhorst, Jana Germeshausen, Manuela Brandes, Gudrun Lee-Gossler, Jacqueline Noyan, Fatih Gatzke, Anna-Katherina Minkov, Milen Greil, Johann Kratz, Christian Petropoulou, Theoni Pellier, Isabelle Bellanné-Chantelot, Christine Rezaei, Nima Mönkemöller, Kirsten Irani-Hakimeh, Noha Bakker, Hans Gerardy-Schahn, Rita Zeidler, Cornelia Grimbacher, Bodo Welte, Karl Klein, Christoph N. Engl. J. Med. 360:32-43 Reactome Database ID Release 81 10306271 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10306271 Reactome R-SSC-70263 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70263.1 GENE ONTOLOGY GO:0006094 The reactions of gluconeogenesis convert mitochondrial pyruvate to cytosolic glucose 6-phosphate which in turn can be hydrolyzed to glucose and exported from the cell. Gluconeogenesis is confined to cells of the liver and kidney and enables glucose synthesis from molecules such as lactate and alanine and other amino acids when exogenous glucose is not available (reviewed, e.g., by Gerich 1993). The process of gluconeogenesis as diagrammed below occurs in two parts: a network of reactions converts mitochondrial pyruvate to cytosolic phosphoenolpyruvate; then phosphoenolpyruvate is converted to glucose 6-phosphate in a single sequence of cytosolic reactions.<p>Three variants of the first part of the process are physiologically important. 1) A series of transport and transamination reactions convert mitochondrial oxaloacetate to cytosolic oxaloacetate which is converted to phosphoenolpyruvate by a hormonally regulated, cytosolic isoform of phosphoenolpyruvate carboxykinase. This variant allows regulated glucose synthesis from lactate. 2) Mitochondrial oxaloacetate is reduced to malate, which is exported to the cytosol and re-oxidized to oxaloacetate. This variant provides reducing equivalents to the cytosol, needed for glucose synthesis from amino acids such as alanine and glutamine. 3) Constitutively expressed mitochondrial phosphoenolpyruvate carboxykinase catalyzes the conversion of mitochondrial oxaloacetate to phosphoenolpyruvate which is then transported to the cytosol. The exact path followed by any one molecule of pyruvate through this reaction network is determined by the tissue in which the reactions are occurring, the source of the pyruvate, and the physiological stress that triggered gluconeogenesis.<p>In all cases, the synthesis of glucose from two molecules of pyruvate requires the generation and consumption of two reducing equivalents as cytosolic NADH + H+. For pyruvate derived from lactate (variants 1 and 3), NADH + H+ is generated with the oxidation of lactate to pyruvate in the cytosol (a reaction of pyruvate metabolism not shown in the diagram). For pyruvate derived from amino acids (variant 2), mitochondrial NADH + H+ generated by glutamate dehydrogenase (a reaction of amino acid metabolism, not shown) is used to reduce oxaloacetate to malate, which is transported to the cytosol and re-oxidized, generating cytosolic NADH + H+. The synthesis of glucose from pyruvate also requires the consumption of six high-energy phosphates, four from ATP and two from GTP.<p>In the second part of gluconeogenesis, cytosolic phosphoenolpyruvate, however derived, is converted to fructose 1,6-bisphosphate by reactions that are the reverse of steps of glycolysis. Hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate is catalyzed by fructose 1,6-bisphosphatase, and fructose 6-phosphate is reversibly isomerized to glucose 6-phosphate. 8379904 Pubmed 1993 Control of glycaemia Gerich, JE Baillieres Clin Endocrinol Metab 7:551-86 Glycolysis 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-RIGHT 2.7.1.2 HK1,2,3,GCK phosphorylate Glc to form G6P alpha-D-Glucose + ATP => alpha-D-glucose 6-phosphate + ADP 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> ACTIVATION Converted from EntitySet in Reactome GCK, HK1, HK2, HK3 glucokinase and hexokinases Reactome DB_ID: 10207415 HK1 HK1 A0A5G2R9N0 Reactome DB_ID: 10207405 UniProt:A0A5G2R9N0 HK1 UniProt A0A5G2R9N0 1 EQUAL 917 EQUAL Reactome Database ID Release 81 10207405 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207405 Reactome R-SSC-70395 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70395.1 HK2 HK2 F1SNW8 Reactome DB_ID: 10207409 UniProt:F1SNW8 HK2 UniProt F1SNW8 1 EQUAL 917 EQUAL Reactome Database ID Release 81 10207409 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207409 Reactome R-SSC-70378 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70378.1 HK3 HK3 K7GS73 Reactome DB_ID: 10207413 UniProt:K7GS73 HK3 UniProt K7GS73 1 EQUAL 923 EQUAL Reactome Database ID Release 81 10207413 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207413 Reactome R-SSC-70412 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70412.1 Reactome Database ID Release 81 10207415 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207415 Reactome R-SSC-450097 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-450097.1 GENE ONTOLOGY GO:0004340 Reactome Database ID Release 81 10207416 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207416 Reactome Database ID Release 81 10207418 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207418 Reactome R-SSC-70420 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70420.1 Cytosolic glucokinase and the three isoforms of hexokinase catalyze the irreversible reaction of glucose and ATP to form glucose 6 phosphate and ADP. In the body glucokinase is found only in hepatocytes and pancreatic beta cells. Glucokinase and the hexokinase enzymes differ in that glucokinase has a higher Km than the hexokinases and is less readily inhibited by the reaction product. As a result, glucokinase should be inactive in the fasting state when glucose concentrations are low but in the fed state should have an activity proportional to glucose concentration. These features are thought to enable efficient glucose uptake and retention in the liver, and to function as a sensor of glucose concentration coupled to insulin release in pancreatic beta cells (Thorens 2001). Glucokinase mutations are associated with MODY2, a heritable early onset form of type II diabetes (Tanizawa et al. 1991; Takeda et al. 1993). Three human hexokinase enzymes have been characterized, HK1 (Aleshin et al. 1998), HK2 (Lehto et al. 1995), and HK3 (Rijksen at al. 1982). 7150652 Pubmed 1982 Compartmentation of hexokinase in human blood cells. Characterization of soluble and particulate enzymes. Rijksen, G Staal, GE Beks, PJ Streefkerk, M Akkerman, Jan Willem N Biochim Biophys Acta 719:431-7 8325892 Pubmed 1993 Structure/function studies of human beta-cell glucokinase. Enzymatic properties of a sequence polymorphism, mutations associated with diabetes, and other site-directed mutants. Takeda, J Gidh-Jain, M Xu, LZ Froguel, P Velho, G Vaxillaire, M Cohen, D Shimada, F Makino, H Nishi, S Stoffel, M Vionnet, N St. Charles, R Harrison, RW Weber, IT Bell, Graeme I Pilkis, SJ J Biol Chem 268:15200-4 1871135 Pubmed 1991 Human liver glucokinase gene: cloning and sequence determination of two alternatively spliced cDNAs Tanizawa, Y Koranyi, LI Welling, CM Permutt, MA Proc Natl Acad Sci U S A 88:7294-7 11780755 Pubmed 2001 GLUT2 in pancreatic and extra-pancreatic gluco-detection (review) Thorens, B Mol Membr Biol 18:265-73 8786021 Pubmed 1995 Human hexokinase II gene: exon-intron organization, mutation screening in NIDDM, and its relationship to muscle hexokinase activity Lehto, M Huang, X Davis, EM Le Beau, MM Laurila, E Eriksson, KF Bell, Graeme I Groop, LC Diabetologia 38:1466-74 9493266 Pubmed 1998 The mechanism of regulation of hexokinase: new insights from the crystal structure of recombinant human brain hexokinase complexed with glucose and glucose-6-phosphate Aleshin, AE Zeng, C Bourenkov, GP Bartunik, HD Fromm, HJ Honzatko, RB Structure 6:39-50 LEFT-TO-RIGHT 2.7.1.147 ADPGK:Mg2+ phosphorylates Glc to G6P 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> ACTIVATION activeUnit: #Protein72 ADPGK:Mg2+ Reactome DB_ID: 10280517 ADPGK ADPGK A0A5G2QK01 Reactome DB_ID: 10280515 UniProt:A0A5G2QK01 ADPGK UniProt A0A5G2QK01 23 EQUAL 497 EQUAL Reactome Database ID Release 81 10280515 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10280515 Reactome R-SSC-5696062 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-5696062.1 1 Mg2+ Magnesium magnesium(2+) Mg++ Reactome DB_ID: 217314 Reactome Database ID Release 81 217314 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=217314 Reactome R-ALL-217314 3 Reactome stable identifier. Use this 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-217314.3 COMPOUND C00305 1 Reactome Database ID Release 81 10280517 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10280517 Reactome R-SSC-5696043 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-5696043.1 GENE ONTOLOGY GO:0043843 Reactome Database ID Release 81 10280518 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10280518 Reactome Database ID Release 81 10280520 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10280520 Reactome R-SSC-5696021 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-5696021.1 GENE ONTOLOGY GO:0061620 Glucose phosphorylation is a central event in cellular metabolism. ADP-dependent glucokinase (ADPGK) can phosphorylate glucose (Glc) using ADP as the phosphate donor to glucose 6-phopshate (G6P). To date, it has not been established whether this phosphorylation supports a significant role in priming glucose for a metabolic fate other than glycolysis (Richter et al. 2012). Stdies of metabolic changes during T cell activation suggest a role for it there (Kaminski et al. 2012). 22219026 Pubmed 2012 Expression and role in glycolysis of human ADP-dependent glucokinase Richter, Susan Richter, Jan P Mehta, Sunali Y Gribble, Amanda M Sutherland-Smith, Andrew J Stowell, Kathryn M Print, Cristin G Ronimus, Ron S Wilson, William R Mol. Cell. Biochem. 364:131-45 23168256 Pubmed 2012 T cell activation is driven by an ADP-dependent glucokinase linking enhanced glycolysis with mitochondrial reactive oxygen species generation Kamiński, Marcin M Sauer, Sven W Kamiński, Marian Opp, Silvana Ruppert, Thorsten Grigaravičius, Paulius Grudnik, Przemysław Gröne, Hermann-Josef Krammer, Peter H Gülow, Karsten Cell Rep 2:1300-15 LEFT-TO-RIGHT 5.3.1.9 alpha-D-glucose 6-phosphate <=> D-fructose 6-phosphate Glucose 6-phosphate is isomerized to form fructose-6-phosphate 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> ACTIVATION Reactome Database ID Release 81 10207488 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10207488 Reactome R-SSC-70471 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SSC-70471.1 Cytosolic phosphoglucose isomerase catalyzes the reversible interconversion of glucose 6-phosphate and fructose 6-phosphate (Tsuboi et al. 1958; Noltmann 1972; Bloxham and Lardy 1973). The active form of the enzyme is a homodimer (Read et al. 2001). Mutations in the enzyme are associated with hemolytic anemia (Xu and Beutler 1994). 0121227022 ISBN 1973 Phosphofructokinase Bloxham, DP Lardy, HA The Enzymes, 3rd ed (Book): 239-278 11371164 Pubmed 2001 The crystal structure of human phosphoglucose isomerase at 1.6 A resolution: implications for catalytic mechanism, cytokine activity and haemolytic anaemia Read, J Pearce, J Li, X Muirhead, H Chirgwin, J Davies, C J Mol Biol 309:447-63 LEFT-TO-RIGHT 3.5.99.6 GNPDA1,2 hexamers deaminate GlcN6P to Fru(6)P