Gene: [00.0/ACADB] 2-Methylacyl-CoA dehydrogenase (of branched-chain fatty acids);


FUN

[1] This dehydrogenase is involved in beta-oxidation of unsaturated branched-chain fatty acids containing the methyl (CH3) group at the alpha (No.2) carbon.
[2] Systematic name: 2-Methylbutanoyl-CoA:(acceptor) oxidoreductase.
[3] The reaction catalyzed: 2-Methylbutanoyl-CoA + acceptor = = 2-methylbut-2-enoyl-CoA + reduced acceptor."

REF

IDN,FUN "Ikeda Y &: JBC, 258, N2, 1066-1076, 1983
PEP,MEB "Tanaka K &: Enzyme, 38, 91-107, 1987

KEY

neu, mtbd, mito

CLA

coding, basic

LOC

RS

MIM

?

EZN

ENZYME: 1.3.99.12

Смотрите также:

  • Gene: [01p31/ACADM] acyl-CoA dehydrogenase M (medium straight-chain fa C4/C12); hypoglycemia, non-ketotic, and secondary carnitine deficiency; dicarboxylicaciduria (MCAD deficiency); Reye-like syndrome (fatty acids beta-oxidation familial d