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

Long-chain specific acyl-CoA dehydrogenase (mitochondrial), encoded by the ACADL gene, is a mitochondrial flavoenzyme that catalyzes the initial dehydrogenation step in the beta-oxidation of long-chain fatty acids (typically C12–C16 acyl-CoA), an essential process for energy production from fat. It is one of four key mitochondrial enzymes performing this function. ACADL deficiency is a rare inborn error of metabolism characterized by impaired fatty acid oxidation, nonketotic hypoglycemia, and increased risk for hepatic and cardiac dysfunction. Regulation of ACADL is important for lipid turnover and systemic energy homeostasis; its dysfunction or altered expression is implicated in metabolic diseases and, potentially, in cancer biology via interaction with the Hippo/YAP pathway. Pharmacological induction (e.g., via PPARα agonists such as fenofibrate) increases ACADL expression and activity, promoting lipid catabolism, while animal studies suggest roles in cardiac and pulmonary health[1][3][4][5].

Other names

Long-chain acyl-CoA dehydrogenaseacyl-CoA dehydrogenase long chainLCADACAD4acyl-Coenzyme A dehydrogenase, long chain

Pharmacological upregulation (e.g., fenofibrate promotes ACADL transcription, enhancing fatty acid catabolism); Targeting Hippo/YAP pathway in cancer (restoration suppresses YAP activity in HCC cells).