EC - Isovaleryl-CoA dehydrogenase

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IntEnz Enzyme Nomenclature


Accepted name:
isovaleryl-CoA dehydrogenase
Other names:
isovaleroyl-coenzyme A dehydrogenase
isovaleryl-coenzyme A dehydrogenase
3-methylbutanoyl-CoA:(acceptor) oxidoreductase
Systematic name:
3-methylbutanoyl-CoA:electron-transfer flavoprotein oxidoreductase




Contains FAD as prosthetic group. Pentanoate can act as donor.

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Protein domains and families: PROSITE:PDOC00070
Structural data: CSA , EC2PDB
Gene Ontology: GO:0008470
CAS Registry Number: 37274-61-6
UniProtKB/Swiss-Prot: (10) [show] [UniProt]


  1. Bachhawat, B.K., Robinson, W.G. and Coon, M.J.
    Enzymatic carboxylation of β-hydroxyisovaleryl coenzyme A.
    J. Biol. Chem. 219 : 539-550 (1956). [PMID: 13319276]
  2. Ikeda, Y. and Tanaka, K.
    Purification and characterization of isovaleryl coenzyme A dehydrogenase from rat liver mitochondria.
    J. Biol. Chem. 258 : 1077-1085 (1983). [PMID: 6401713]
  3. Tanaka, K., Budd, M.A., Efron, M.L. and Isselbacher, K.J.
    Isovaleric acidemia: a new genetic defect of leucine metabolism.
    Proc. Natl. Acad. Sci. USA 56 : 236-242 (1966). [PMID: 5229850]

[EC created 1978 as EC, modified 1986, transferred 2012 to EC]