ACADL Proteins and Enzymes

The protein encoded by the ACADL gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrialflavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymesthat catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene arethe cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. (provided byRefSeq)