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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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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

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

  1. 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. 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: a new inborn error of L-isoleucine metabolism. Pediatr Res, 2000