The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms.
ACADVL; acyl-CoA dehydrogenase, very long chain; acyl Coenzyme A dehydrogenase, very long chain; very long-chain specific acyl-CoA dehydrogenase, mitochondrial; ACAD6; LCACD; VLCAD; acyl-Coenzyme A dehydrogenase, very long chain; fb52d04; zgc:64067; wu:fb52d04; wu:fc75e01