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).