ACADL

ACADL 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 be

Acyl-CoA Dehydrogenase Long Chain

Long-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats (By similarity). The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA (By similarity). Among the different mitochondrial acyl-CoA dehydrogenases, long-chain specific acyl-CoA dehydrogenase can act on saturated and unsaturated acyl-CoAs with 6 to 24 carbons with a preference for 8 to 18 carbons long primary chains.

Carnitine catabolic process
Carnitine metabolic process, CoA-linked
Cellular lipid catabolic process
Fatty acid beta-oxidation
Fatty acid beta-oxidation using acyl-CoA dehydrogenase
Long-chain fatty acid catabolic process
Negative regulation of fatty acid biosynthetic process
Negative regulation of fatty acid oxidation
Positive regulation of cold-induced thermogenesis
Regulation of cholesterol metabolic process
Temperature homeostasis

Mitochondrion matrix

Acetylation at Lys-318 and Lys-322 in proximity of the cofactor-binding sites strongly reduces catalytic activity. These sites are deacetylated by SIRT3.