Acyl Coenzyme A Dehydrogenase, Long Chain (ACADL)

ACADL is a protein 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.

Impaired ACADL activity results in non-ketotic hypoglycemia, hypotonia, muscle weakness and episodes of cardiorespiratory arrest associated with fasting. A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.