Protein Name acyl-CoA dehydrogenase very long chain
Organism Homo sapiens
Gene ID 37
Gene Symbol

ACADVL

UniProt P49748 (ACADV_HUMAN), B3KPA6 (B3KPA6_HUMAN)
Relationships Total Number of functionally related compound(s) : 58
Total Number of Articles : 74
Description

acyl-CoA dehydrogenase very long chain

Gene Summary

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. [provided by RefSeq, Jul 2008]

synonyms
  • very long-chain specific acyl-CoA dehydrogenase, mitochondrial
  • acyl-Coenzyme A dehydrogenase, very long chain
  • ACAD6
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Properties