Carnitine palmitoyltransferase II

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

Function
Carnitine palmitoyltransferase II precursor (CPT2) is a nuclear protein which is transported to the is_associated_with::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 is_associated_with::carnitine palmitoyltransferase II deficiency.



Model organisms
is_associated_with::Model organisms have been used in the study of CPT2 function. A conditional is_associated_with::knockout mouse line called Cpt2tm1b(KOMP)Wtsi was generated at the is_associated_with::Wellcome Trust Sanger Institute. Male and female animals underwent a standardized is_associated_with::phenotypic screen to determine the effects of deletion. Additional screens performed: - In-depth immunological phenotyping