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ACADVLA gene on chromosome 17p13.1 that encodes acyl-Coenzyme A dehydrogenase, a protein bound to the inner mitochondrial membrane that catalyses the first step of the mitochondrial fatty acid beta-oxidation pathway; it is specific to long-chain and very long-chain fatty acids.
ACADVL mutation reduces myocardial fatty acid beta-oxidation and is linked to cardiomyopathy and VLCAD—Very long-chain acyl-CoA dehydrogenase—deficiency.
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