ACADVL 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 its gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.