Carnitine O-palmitoyltransferase 1 (CPT1)


UniProt Number: P50416
Alternate Names: Carnitine O-palmitoyltransferase I, liver isoform,CPT I, CPTI-L, Carnitine palmitoyltransferase 1A
Structure and Function: The carnitine palmitoyltransferase system is an essential step in the beta-oxidation of long chain fatty acids. This transfer system is necessary because, while fatty acids are activated (in the form of a thioester linkage to coenzyme A) on the outer mitochondrial membrane, the activated fatty acids are oxidized within the mitochondrial matrix. Long chain fatty acids such as palmitoyl-CoA, unlike short- and medium-chain fatty acids, cannot freely diffuse through the mitochondrial inner membrane, and require a shuttle system to be transported to the mitochondrial matrix. Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system, catalyzing the transfer of the acyl group from coenzyme A to carnitine to form palmitoylcarnitine. A translocase then shuttles the acyl carnitine across the inner mitochondrial membrane where it is converted back into palmitoyl-CoA. By acting as an acyl group acceptor, carnitine may also play the role of regulating the intracellular CoA:acy-CoA ratio
Disease Associations: Defects in CPT1A are the cause of carnitine palmitoyltransferase 1A deficiency (CPT1AD) [MIM:255120]; also known as CPT-I deficiency or CPT1A deficiency. CPT1AD is a rare autosomal recessive metabolic disorder of long-chain fatty acid oxidation characterized by severe episodes of hypoketotic hypoglycemia usually occurring after fasting or illness. Onset is in infancy or early childhood.


Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount
ab128568 CPT1A Antibody Human, Mouse, Rat WB, ICC/IF, In-Cell ELISA 100 µg


Proteins
Cat. No. Name Source Amount
ab128569 CPT1A protein (His tag) Human 50 µg



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