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.
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Protein Aliases: acyl-Coenzyme A dehydrogenase, very long chain; Very long-chain specific acyl-CoA dehydrogenase, mitochondrial; VLCAD
Gene Aliases: ACAD6; ACADVL; LCACD; VLCAD
UniProt ID: (Human) P49748
Entrez Gene ID: (Human) 37