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

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

Stable Identifier

R-HSA-70745

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

3-methylbutanoyl-CoA + H(+) + oxidized (electron-transfer flavoprotein) => 3-methyl-(2E)-butenoyl-CoA + reduced (electron-transfer flavoprotein)

ReviewStatus

5/5

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

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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 existence of a tetramer of IVD polypeptides lacking an amino-terminal 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