CPT-1

Carnitine palmitoyltransferase-1 (CPT-1) is the enzyme that catalyzes the rate limiting step in fatty acid oxidation of palmitoyl; the transfer of the 16 carbon fatty acids into the mitochondrion matrix. Inside the mitochondrion the fatty acid undergoes ß-oxidation. To transfer the fatty acid into the mitochondrion, CPT-1 attaches the acyl group of the fatty acid to the hydroxyl group of carnitine (Figure 3a) . The resulting ester enters the mitochondrion through facilitated diffusion through the acyl-carnitine/carnitine transporter. Once it is on the on matrix side of the transporter, carnitine palmitoyltransferase-II catalyses the removal of the fatty acid from the carnitine. Figure 3b shows a schematic representation of how acyl groups are transferred to the mitochondrial matrix via CPT-1.