FEATURES

FAMILY

DESCRIPTION

Also known as ACADV_HUMAN, ACADVL, VLCAD. Very long-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats (PubMed:7668252, PubMed:9461620, PubMed:18227065, PubMed:9839948, PubMed:9599005). The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA (PubMed:7668252, PubMed:9461620, PubMed:18227065, PubMed:9839948). Among the different mitochondrial acyl-CoA dehydrogenases, very long-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 12 to 24 carbons long primary chains (PubMed:21237683, PubMed:9839948). Homodimer (PubMed:9461620, PubMed:18227065, PubMed:9599005). Homodimerizes after import into the mitochondrion (PubMed:9599005).

EXTERNAL LINKS

ACADVL

Very long-chain specific acyl-CoA dehydrogenase, mitochondrial

ACADVL

Molecular Synopsis