carnitine palmitoyltransferase 1 (CPT1)

Function: - CPT1 transfers acyl groups of long chain fatty acids from CoA to carnitine at the cytoplasmic surface of the inner mitochondrial membrane - CPT2 transfers the acyl group back to CoA on the matrix surface of the membrane - 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. Laboratory: - carnitine palmitoyltransferase 1 in fibroblasts - carnitine palmitoyltransferase 1 in leukocytes

Specific

carnitine palmitoyltransferase-1A, liver isoform (CPT1A, CPT1) carnitine palmitoyltransferase-1B, muscle isoform (CPT1B, KIAA1670)

Properties

COMPARTMENT: inner mitochondrial membrane MOTIF: membrane region active site

References

Textbook of Biochemistry with Clinical Correlations, 3rd ed., TM Devlin (ed), Wiley-Liss, NY 1992 pg 408