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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.
Gene Name: | acyl-CoA dehydrogenase, very long chain |
Synonyms: | ACADVL, ACAD6, LCACD, VLCAD |
Target Sequences: | NM_000018 NP_000009.1 P49748 |
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