acyl-CoA dehydrogenase, very long chain


ACADVL (may also be known as: None)




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]


acdh-12 Caenorhabditis elegans
CG7461 Drosophila melanogaster
acadvl Danio rerio
Acadvl Mus musculus
Acadvl Rattus norvegicus

Links to external resources

Changes associated with this gene

GO Terms

GO IDGO TermGO Category
GO:0001659 temperature homeostasis biological_process
GO:0006631 fatty acid metabolic process biological_process
GO:0006635 fatty acid beta-oxidation biological_process
GO:0006987 activation of signaling protein activity involved in unfolded protein response biological_process
GO:0015980 energy derivation by oxidation of organic compounds biological_process
GO:0030968 endoplasmic reticulum unfolded protein response biological_process
GO:0033539 fatty acid beta-oxidation using acyl-CoA dehydrogenase biological_process
GO:0042760 very long-chain fatty acid catabolic process biological_process
GO:0044255 cellular lipid metabolic process biological_process
GO:0044281 small molecule metabolic process biological_process
GO:0045717 negative regulation of fatty acid biosynthetic process biological_process
GO:0046322 negative regulation of fatty acid oxidation biological_process
GO:0055114 oxidation-reduction process biological_process
GO:0090181 regulation of cholesterol metabolic process biological_process
GO:0005739 mitochondrion cellular_component
GO:0005743 mitochondrial inner membrane cellular_component
GO:0005759 mitochondrial matrix cellular_component
GO:0016020 membrane cellular_component
GO:0042645 mitochondrial nucleoid cellular_component
GO:0000062 fatty-acyl-CoA binding molecular_function
GO:0003995 acyl-CoA dehydrogenase activity molecular_function
GO:0004466 long-chain-acyl-CoA dehydrogenase activity molecular_function
GO:0017099 very-long-chain-acyl-CoA dehydrogenase activity molecular_function
GO:0050660 flavin adenine dinucleotide binding molecular_function