Mitochondrial Carnitine O-palmitoyltransferase 1A (None)

Mitochondrial carnitine O-palmitoyltransferase 1A (CPT1A) is a key enzyme located on the outer mitochondrial membrane. It catalyzes the transfer of long-chain fatty acyl groups from coenzyme A to L-carnitine, forming acylcarnitines. This reaction is essential for transporting long-chain fatty acids into mitochondria for β-oxidation, making CPT1A a rate-limiting step in mitochondrial fatty acid oxidation. CPT1A is predominantly expressed in liver, kidney, pancreas, adipose tissue, lymphocytes, and fibroblasts. It is inhibited by malonyl-CoA, providing tight regulation between lipid synthesis and degradation pathways. Deficiency or dysfunction of CPT1A impairs hepatic ketogenesis and energy production from fats, especially during fasting, and can result in hypoketotic hypoglycemia. Altered expression or localization has been observed in various cancers.

Other names

Carnitine O-palmitoyltransferase 1, liver isoformL-CPT-1CPT1A

Catalyzes transfer of long-chain acyl groups from Coenzyme A to carnitine, enabling transport of activated fatty acids into mitochondria for β-oxidation. Inhibition by malonyl-CoA regulates lipid synthesis and degradation.