Carnitine Palmitoyltransferase 2, Mitochondrial (CPT2)

CPTASE; Carnitine O-Palmitoyltransferase 2; Carnitine palmitoyltransferase II

Carnitine Palmitoyltransferase 2, Mitochondrial (CPT2)

Carnitine palmitoyltransferase II precursor (CPT2) is a nuclear protein which is transported to the mitochondrial inner membrane. CPT2 together with carnitine palmitoyltransferase I oxidizes long-chain fatty acids in the mitochondria. Defects in this gene are associated with mitochondrial long-chain fatty-acid (LCFA) oxidation disorders and carnitine palmitoyltransferase II deficiency.

The carnitine palmitoyltransferase (CPT) enzyme system, in conjunction with acyl-CoA synthetase and carnitine/acylcarnitine translocase, provides the mechanism whereby long-chain fatty acids are transferred from the cytosol to the mitochondrial matrix to undergo beta-oxidation.

Organism species: Homo sapiens (Human)

Organism species: Mus musculus (Mouse)

Organism species: Rattus norvegicus (Rat)