Carnitine palmitoyltransferase system (mainly Carnitine palmitoyltransferase 1 and 2) (CPT system (CPT1, CPT2))

Fatty acid mitochondrial transport refers to the tightly regulated process by which long-chain fatty acids are transported from the cytosol into mitochondria, where they undergo β-oxidation to provide energy for the cell. This transport is mediated primarily by the carnitine palmitoyltransferase (CPT) system, which includes CPT1 on the outer mitochondrial membrane, carnitine-acylcarnitine translocase (CACT) in the inner membrane, and CPT2 on the inner mitochondrial membrane. These enzymes work together to shuttle fatty acyl-CoA derivatives across mitochondrial membranes by converting them to acylcarnitines, allowing passage, and then reconverting them back to fatty acyl-CoA inside the matrix. This process is essential in tissues with high-energy demands such as heart, skeletal muscle, and liver, and is regulated by nutritional status, hormonal signaling, and intracellular energy needs. Genetic disorders or pharmacologic inhibition of this pathway can lead to severe metabolic disturbances[2][3][1].

Other names

Carnitine shuttleCPT systemMitochondrial fatty acid importFatty acid β-oxidation entrance

Inhibition of carnitine palmitoyltransferase 1 reduces mitochondrial fatty acid uptake, lowering β-oxidation rates\nModulation of carnitine availability alters fatty acid flux into mitochondria