BioPAX pathway converted from "isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2" in the Reactome database.1.3.8.4isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2Mitochondrial isovaleryl dehydrogenase (IVD) catalyzes the reaction of isovaleryl-CoA and FAD to form beta-methylcrotonyl-CoA and FADH2 (Finocchiaro et al. 1978; Rhead and Tanaka 1980). Crystallographic studies demonstrated the existene of a tetramer of IVD polypeptides lacking an aminoterminal mitochondrial targeting sequence (Tiffany et al. 1997).Reactome DB_ID: 1135961mitochondrial matrixGO0005759FAD [ChEBI:16238]FADFlavin adenine dinucleotideReactomehttp://www.reactome.orgChEBI16238Reactome DB_ID: 707151isovaleryl-CoA [ChEBI:15487]isovaleryl-CoAChEBI15487Reactome DB_ID: 7074413-methylbut-2-enoyl-CoA [ChEBI:15486]3-methylbut-2-enoyl-CoAChEBI15486Reactome DB_ID: 316491FADH2 [ChEBI:17877]FADH2ChEBI17877PHYSIOL-LEFT-TO-RIGHTACTIVATIONReactome DB_ID: 70730IVD tetramer [mitochondrial matrix]IVD tetramerisovaleryl-CoA dehydrogenase tetramerReactome DB_ID: 707294UniProt:P26440 IVDIVDIVDFUNCTION Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA (PubMed:7640268).PATHWAY Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 1/3.SUBUNIT Homotetramer.SIMILARITY Belongs to the acyl-CoA dehydrogenase family.CAUTION It is uncertain whether Met-1 or Met-4 is the initiator.Homo sapiensNCBI Taxonomy9606UniProtP26440Chain Coordinates30EQUAL423EQUALReactome DB_ID: 1135964Reactome Database ID Release 7570730Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=70730ReactomeR-HSA-707301Reactome stable identifier. Use this 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-70730.1GO0008470GO molecular functionReactome Database ID Release 7570743Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=70743Reactome Database ID Release 7570745Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=70745ReactomeR-HSA-707453Reactome stable identifier. Use this 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-70745.39214289Pubmed1997Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,.Tiffany, KARoberts, DLWang, MPaschke, RMohsen, AWVockley, JKim, Jung-Ja PBiochemistry 36:8455-643597357Pubmed1987Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver.Finocchiaro, GIto, MTanaka, KJ Biol Chem 262:7982-96928646Pubmed1980Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia.Rhead, WJTanaka, KProc Natl Acad Sci U S A 77:580-3