isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2

Stable Identifier
Reaction [transition]
Homo sapiens
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Mitochondrial 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).

Literature References
PubMed ID Title Journal Year
6928646 Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia.

Rhead, WJ, Tanaka, K

Proc Natl Acad Sci U S A 1980
9214289 Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,.

Wang, M, Paschke, R, Tiffany, KA, Roberts, DL, Kim, JJ, Mohsen, AW, Vockley, J

Biochemistry 1997
3597357 Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver.

Tanaka, K, Finocchiaro, G, Ito, M

J Biol Chem 1987
Catalyst Activity

isovaleryl-CoA dehydrogenase activity of IVD tetramer [mitochondrial matrix]

Orthologous Events
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