BioPAX pathway converted from "ALAS condenses SUCC-CoA and Gly to form dALA" in the Reactome database.LEFT-TO-RIGHT2.3.1.37ALAS condenses SUCC-CoA and Gly to form dALASuccinyl CoA and glycine condense to form 5-aminolevulinate (ALA)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>SUCC-CoASuccinyl coenzyme Asuccinyl-CoA(5-)Reactome DB_ID: 70958mitochondrial matrixGENE ONTOLOGYGO:0005759succinyl-CoA(5-) [ChEBI:57292]succinyl-CoA(5-)S-(3-carboxy-propionyl)-CoA(5-)succinyl-coenzyme A(5-)3'-phosphonatoadenosine 5'-{3-[(3R)-4-{[3-({2-[(3-carboxylatopropanoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-3-hydroxy-2,2-dimethyl-4-oxobutyl]diphosphate}succinyl-CoAS-(hydrogen succinyl)CoA(5-)succinyl-CoA pentaanionChEBICHEBI:57292Reactome Database ID Release 7570958Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=70958ReactomeR-ALL-709584Reactome stable identifier. Use this 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-70958.4Reactomehttp://www.reactome.orgGlyAminoacetic acidglycine zwitterionglycineReactome DB_ID: 159549glycine zwitterion [ChEBI:57305]glycine zwitterionglycineDHMQDGOQFOQNFH-UHFFFAOYSA-N2-azaniumylacetateC2H5NO2InChI=1S/C2H5NO2/c3-1-2(4)5/h1,3H2,(H,4,5)[NH3+]CC([O-])=O75.06660ChEBICHEBI:57305Reactome Database ID Release 75159549Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=159549ReactomeR-ALL-1595494Reactome stable identifier. Use this 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-159549.4COMPOUNDC00037additional informationMIMI:0361H+hydronReactome DB_ID: 113529hydron [ChEBI:15378]hydronChEBICHEBI:15378Reactome Database ID Release 75113529Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113529ReactomeR-ALL-1135294Reactome stable identifier. Use this 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-113529.4COMPOUNDC00080dALA5-aminolevulinate5-aminolevulinic aciddelta-Aminolevulinic acid5-ammoniolevulinateReactome DB_ID: 1894665-ammoniolevulinate [ChEBI:356416]5-ammoniolevulinate5-azaniumyl-4-oxopentanoate5-ammonio-4-oxovalerate5-aminolevulinate5-ammonio-4-oxopentanoateChEBICHEBI:356416Reactome Database ID Release 75189466Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=189466ReactomeR-ALL-1894664Reactome stable identifier. Use this 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-189466.4CoACoA-SHcoenzyme AReactome DB_ID: 29374coenzyme A [ChEBI:15346]coenzyme AChEBICHEBI:15346Reactome Database ID Release 7529374Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29374ReactomeR-ALL-293743Reactome stable identifier. Use this 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-29374.3COMPOUNDC00010CO2carbon dioxideReactome DB_ID: 29376carbon dioxide [ChEBI:16526]carbon dioxideChEBICHEBI:16526Reactome Database ID Release 7529376Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=29376ReactomeR-ALL-293763Reactome stable identifier. Use this 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-29376.3COMPOUNDC00011ACTIVATIONConverted from EntitySet in ReactomeALAS1,2Reactome DB_ID: 10705260ALAS1 homodimer (pyridoxal phosphate cofactor)Reactome DB_ID: 10705253hem1PXLP-ALAS1O14092Reactome DB_ID: 10705251UniProt:O14092hem1Schizosaccharomyces pombeNCBI Taxonomy4896UniProtO14092N6-pyridoxal phosphate-L-lysineMODMOD:0012857EQUAL640EQUALReactome Database ID Release 7510705251Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10705251ReactomeR-SPO-10220861Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SPO-1022086.12Reactome Database ID Release 7510705253Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10705253ReactomeR-SPO-1894461Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SPO-189446.1ALAS2 homodimer (pyridoxal phosphate cofactor)Reactome DB_ID: 10705258hem1PXLP-K391-ALAS2O14092Reactome DB_ID: 10705256391EQUAL50EQUAL587EQUALReactome Database ID Release 7510705256Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10705256ReactomeR-SPO-10220831Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SPO-1022083.12Reactome Database ID Release 7510705258Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10705258ReactomeR-SPO-1894431Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SPO-189443.1Reactome Database ID Release 7510705260Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10705260ReactomeR-SPO-1894291Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SPO-189429.1GENE ONTOLOGYGO:0003870gene ontology term for cellular functionMIMI:0355Same Catalyst ActivityReactome Database ID Release 7510705261Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10705261Reactome Database ID Release 7510705263Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=10705263ReactomeR-SPO-1894421Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SPO-189442.1The committed step for porphyrin synthesis is the formation of 5-aminolevulinate (ALA) by condensation of glycine (from the general amino acid pool) and succinyl-CoA (from the TCA cycle), in the mitochondrial matrix. The reaction is catalyzed by two different ALA synthases, one expressed ubiquitously (ALAS1) and the other only expressed in erythroid precursors (ALAS2). Both enzymes are expressed as homodimers and require pyridoxal 5-phosphate as a cofactor.<br>No disease-causing mutations of ALAS1 have been recognised in humans. Mutations in ALAS2 cause X-linked sideroblastic anaemia (XLSA), characterised by a microcytic hypochromic anaemia.16121195Pubmed2005Crystal structure of 5-aminolevulinate synthase, the first enzyme of heme biosynthesis, and its link to XLSA in humansAstner, ISchulze, JOvan den Heuvel, JJahn, DSchubert, WDHeinz, DWEMBO J 24:3166-771939222Pubmed1991Biosynthesis of delta-aminolevulinic acid and the regulation of heme formation by immature erythroid cells in manGardner, LCSmith, SJCox, Timothy MJ Biol Chem 266:22010-8inferred from electronic annotationEVIDENCE CODEECO:0000203INHIBITIONReactome Database ID Release 75189417Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=189417ReactomeR-HSA-1894171Reactome stable identifier. Use this 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-189417.1hemeprotohemeferroheme bReactome DB_ID: 189444ferroheme b [ChEBI:17627]ferroheme bChEBICHEBI:17627Reactome Database ID Release 75189444Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=189444ReactomeR-ALL-1894443Reactome stable identifier. Use this 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-189444.3COMPOUNDC00032