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

Stable Identifier
R-HSA-70859
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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
12359132 Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans.

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

Mol Genet Metab 2002
9889013 Isolated isobutyryl-CoA dehydrogenase deficiency: an unrecognized defect in human valine metabolism.

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

Mol Genet Metab 1999
14752098 Structures of isobutyryl-CoA dehydrogenase and enzyme-product complex: comparison with isovaleryl- and short-chain acyl-CoA dehydrogenases

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

J Biol Chem 2004
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
acyl-CoA dehydrogenase activity of ACAD8 tetramer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea