alpha-methylbutyryl-CoA + FAD => tiglyl-CoA + FADH2
2MBUT-CoA + FAD + H+ → tiglyl-CoA + FADH2
- Reactome
- R-HSA-70800
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:43781
Inputs
- 2MBUT-CoACHEBI:156439, C26H40N7O17P3S, mitochondrial matrix
- FADCHEBI:57692, C27H30N9O15P2, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
Outputs
- tiglyl-CoACHEBI:57337, C26H38N7O17P3S, mitochondrial matrix
- FADH2CHEBI:58307, C27H33N9O15P2, mitochondrial matrix
Catalysed by
- acyl-CoA dehydrogenase activity of ACADSB tetramer [mitochondrial matrix]EC 1.3.99.3
Literature
- Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism. Am J Hum Genet, 2000
- 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: a new inborn error of L-isoleucine metabolism. Pediatr Res, 2000