3-methyl-2-oxobutanoate dehydrogenase complexes:3-methyl-2-oxobutanoate dehydrogenase
a part of multi-step reaction (see R01701+R02662+R07618, possibly identical to R01210)
subsequently R02662
TPP-dependent enzyme (see R07599+R07600)
Enzymatic reactions [BR:br08201]
1. Oxidoreductase reactions
1.2 Acting on the aldehyde or oxo group of donors
1.2.4 With a disulfide as acceptor
1.2.4.4
R01701 3-Methyl-2-oxobutanoic acid + Enzyme N6-(lipoyl)lysine <=> [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2
3-methyl-2-oxobutanoate dehydrogenase complexes:dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
a part of multi-step reaction (see R01701+R02662+R07618, possibly identical to R01210)
following after R01701
Andresen BS, Christensen E, Corydon TJ, Bross P, Pilgaard B, Wanders RJ, Ruiter JP, Simonsen H, Winter V, Knudsen I, Schroeder LD, Gregersen N, Skovby F
Title
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.