acyl-CoA dehydrogenase, very long chain
Symbol
ACADVL
(may also be known as: None)
Organism
Human
Description
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]
Orthologs
| Symbol | Species | |
|---|---|---|
| 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
| Identifier | Name | Type | Tissues | Organism | Gene | Data | Actions |
|---|---|---|---|---|---|---|---|
| DAA2778 | Gene expression of ACADVL decreases with age | Molecular | adipose tissue | Human | ACADVL | — |
GO Terms
| GO ID | GO Term | GO 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 | |