isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2

Stable Identifier
Reaction [transition]
Homo sapiens
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Mitochondrial isobutyryl-CoA dehydrogenase (ACAD8) catalyzes the reaction of isobutyryl-CoA and FAD to form methacrylyl-CoA and FADH2 (Roe et al. 1999; Nguyen et al. 2002). Crystallographic studies have shown the active enzyme to be a tetramer of ACAD8 polypeptides whose aminoterminal 23 residues, a mitochondrial targeting sequence, have been removed (Bataille et al. 2004).

Literature References
PubMed ID Title Journal Year
9889013 Isolated isobutyryl-CoA dehydrogenase deficiency: an unrecognized defect in human valine metabolism.

Mardach, R, Galindo, A, Cederbaum, SD, Roe, DS, Sweetman, L

Mol Genet Metab 1999
12359132 Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans.

Cederbaum, SD, Lench, NJ, Roe, DS, Corydon, TJ, Ghisla, S, Andresen, BS, Abd-El Razik, N, Mohsen, AW, Vockley, J, Nguyen, TV

Mol Genet Metab 2002
14752098 Structures of isobutyryl-CoA dehydrogenase and enzyme-product complex: comparison with isovaleryl- and short-chain acyl-CoA dehydrogenases

Kim, JJ, Battaile, KP, Vockley, J, Nguyen, TV

J Biol Chem 2004
Catalyst Activity

acyl-CoA dehydrogenase activity of ACAD8 tetramer [mitochondrial matrix]

Orthologous Events
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