Mitochondrial fatty acid beta-oxidation is one of the main energy-producing metabolic pathways in eukaryotes. Acyl-CoA dehydrogenases (ACADs) (EC 1.3.99.13) are mitochondrial enzymes that catalyze the initial rate-limiting step in the beta-oxidation of fatty acyl-CoA.
5 acyl-CoA dehydrogenases (ACADs) had been reported:
short-chain acyl-CoA dehydrogenase (ACADS) (MIM.606885)
medium-chain acyl-CoA dehydrogenase (ACADM) (EC 1.3.99.3)
long-chain acyl-CoA dehydrogenase (ACADL) (MIM.609576)
very long-chain acyl-CoA dehydrogenase (ACADVL) (MIM.609575)
isovaleryl-CoA dehydrogenase (MIM.243500)
2-methyl branched-chain acyl-CoA dehydrogenase.
The first 4 catalyze the initial reaction in the beta-oxidation of fatty acids. The last 2 catalyze the dehydrogenation of branched short-chain acyl-CoAs in the metabolism of the branched-chain amino acids. All 5 may have evolved from a common ancestral gene.
Members
| ACAD1 | ACAD2 | ACAD3 | ACAD4 | ACAD5 | ACAD6 | ACAD7 | ACAD8 | ACAD9 | ACAD10 |
See also
| ACADS (SCAD) | ACADM (MCAD) | ACADL (LCAD) | ACADVL (VLCAD) |
References
He M, Rutledge SL, Kelly DR, Palmer CA, Murdoch G, Majumder N, Nicholls RD, Pei Z, Watkins PA, Vockley J. A new genetic disorder in mitochondrial fatty acid beta-oxidation: ACAD9 deficiency. Am J Hum Genet. 2007 Jul;81(1):87-103. PMID: 17564966
