IUBMB Enzyme Nomenclature


Accepted name: isovaleryl-CoA dehydrogenase

Reaction: isovaleryl-CoA + electron-transfer flavoprotein = 3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein

Other name(s): isovaleryl-coenzyme A dehydrogenase; isovaleroyl-coenzyme A dehydrogenase; 3-methylbutanoyl-CoA:(acceptor) oxidoreductase

Systematic name: 3-methylbutanoyl-CoA:electron-transfer flavoprotein oxidoreductase

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

Links to other databases: BRENDA, EXPASY, KEGG, Metacyc, PDB, CAS registry number: 37274-61-6


1. Bachhawat, B.K., Robinson, W.G. and Coon, M.J. Enzymatic carboxylation of β-hydroxyisovaleryl coenzyme A. J. Biol. Chem. 219 (1956) 539-550. [PMID: 13319276]

2. Ikeda, Y. and Tanaka, K. Purification and characterization of isovaleryl coenzyme A dehydrogenase from rat liver mitochondria. J. Biol. Chem. 258 (1983) 1077-1085. [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 (1966) 236-242. [PMID: 5229850]

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

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