Carnitine palmitoyltransferase II


Carnitine O-palmitoyltransferase 2, mitochondrial is an enzyme that in humans is encoded by the CPT2 gene.

Function

Carnitine palmitoyltransferase II precursor is a mitochondrial membrane 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 oxidation disorders and carnitine palmitoyltransferase II deficiency.

Model organisms

s have been used in the study of CPT2 function. A conditional knockout mouse line called Cpt2tm1bWtsi was generated at the Wellcome Trust Sanger Institute. Male and female animals underwent a standardized phenotypic screen to determine the effects of deletion. Additional screens performed: - In-depth immunological phenotyping