ACAD 6, ACAD6, ACADV_HUMAN, Acadvl, Acyl CoA dehydrogenase very long chain, Acyl Coenzyme A dehydrogenase very long chain, LCACD, mitochondrial, Very long chain specic acyl CoA dehydrogenase, Very long chain specic acyl CoA dehydrogenase mitochondrial, Very long-chain specic acyl-CoA dehydrogenase, VLCAD.
ACADVL (acyl-Coenzyme A dehydrogenase, very long chain) catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. It is specific to esters of long-chain and very long chain fatty acids such as palmitoyl-CoA and stearoyl-CoA. Deficiencies in ACADVL are associated with reduced myocardial fatty acid beta-oxidation and cardiomyopathy.