The protein encoded by this gene is a nuclear protein which is transported to the mitochondrial inner membrane. Together with carnitine palmitoyltransferase I, the encoded protein oxidizes long-chain fatty acids in the mitochondria. Defects in this gene are associated with mitochondrial long-chain fatty-acid (LCFA) oxidation disorders. [provided by RefSeq, Jul 2008],CPT1, CPTASE, IIAE4,Cancer,Cardiovascular,Endocrine & Metabolism,Lipid Metabolism,Lipids,Lipids_Fatty Acids,Mitochondrial metabolism,Mitochondrial metabolism_Mitochondrial markers,Signal Transduction,CPT2