Recombinant Human Long-chain specific acyl-CoA dehydrogenase, mitochondrial (ACADL)

Uniprot Id

P28330

Target Species

Human

Target Name

ACADL

Target Full Name

Long-chain specific acyl-CoA dehydrogenase, mitochondrial

Target Function

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. 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. 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.

Target Involvement

Acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

Acyl-CoA dehydrogenase family

Target Synonyms

ACAD4; ACADL; ACADL_HUMAN; Acyl Coenzyme A dehydrogenase long chain; Acyl-CoA dehydrogenase long chain; FLJ94052; LCAD; Long chain acyl CoA dehydrogenase; Long-chain specific acyl-CoA dehydrogenase; mitochondrial

Target Background

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.