Gene:
acyl-Coenzyme A dehydrogenase, very long chain
Gene ID:
201139
Symbol:
ACADVL
Species:
1000
Description:
The ACADVL gene product is targetted 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. ACADVL deficiency reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.