acyl-CoA dehydrogenase, long chain

Symbol

ACADL (may also be known as: None)

Organism

Human

Description

The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq, Jul 2008]

Orthologs

SymbolSpecies
acadl Danio rerio
Acadl Mus musculus
Acadl 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:0019254 carnitine metabolic process, CoA-linked biological_process
GO:0033539 fatty acid beta-oxidation using acyl-CoA dehydrogenase biological_process
GO:0042413 carnitine catabolic process biological_process
GO:0042758 long-chain fatty acid catabolic process biological_process
GO:0044242 cellular lipid 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:0051289 protein homotetramerization biological_process
GO:0055114 oxidation-reduction process biological_process
GO:0090181 regulation of cholesterol metabolic process biological_process
GO:0005737 cytoplasm cellular_component
GO:0005739 mitochondrion cellular_component
GO:0005759 mitochondrial matrix cellular_component
GO:0031966 mitochondrial membrane 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:0016401 palmitoyl-CoA oxidase activity molecular_function
GO:0050660 flavin adenine dinucleotide binding molecular_function