isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2
ISB-CoA + FAD + H+ → MACR-CoA + FADH2
- Reactome
- R-HSA-70859
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:44181
Inputs
- ISB-CoACHEBI:57338, C25H38N7O17P3S, mitochondrial matrix
- FADCHEBI:57692, C27H30N9O15P2, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
Outputs
- MACR-CoACHEBI:62500, C25H36N7O17P3S, mitochondrial matrix
- FADH2CHEBI:58307, C27H33N9O15P2, mitochondrial matrix
Catalysed by
- acyl-CoA dehydrogenase activity of ACAD8 tetramer [mitochondrial matrix]EC 1.3.99.3
Literature
- Structures of isobutyryl-CoA dehydrogenase and enzyme-product complex: comparison with isovaleryl- and short-chain acyl-CoA dehydrogenases J Biol Chem, 2004
- Isolated isobutyryl-CoA dehydrogenase deficiency: an unrecognized defect in human valine metabolism. Mol Genet Metab, 1999
- Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans. Mol Genet Metab, 2002