carnitine palmitoyltransferase-2 (CPT2)

Function

• fatty acid beta-oxidation cycle
• transfers acyl groups of long chain fatty acids from carnitine to CoA at the matrix surface of the inner mitochondrial membrane
• in contrast, CPT1 transfers the acyl group from CoA to carnitine on the cytoplasmic surface of the membrane

palmitoyl-CoA + L-carnitine <--> CoA + L- palmitoylcarnitine

Structure

• belongs to the carnitine/choline acetyltransferase family

Compartment

mitochondrial inner membrane

Pathology

• defects in CPT2 are the cause of carnitine palmitoyltransferase 2 deficiency

Laboratory

• CPT2 gene mutation

Notes

• carnitine palmitoyl transferases (CPT) function primarily in the mitochondrial transport of fatty acyl CoA's with chain lengths of C12-C18
• shorter chain fatty acids can cross the inner mitochondrial membrane directly & be converted to their CoA derivatives in the mitochondrial matrix
• thus beta oxidation of C4-C10 is carnitine independent

More general terms

• carnitine palmitoyltransferase

Database

• UniProt: http://www.uniprot.org/uniprot/P23786.html
• OMIM: https://mirror.omim.org/entry/600650
• OMIM: https://mirror.omim.org/entry/255110
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=1376