Carnitine palmitoyltransferase I

Carnitine palmitoyltransferase I (CPT1)is a mitochondrial enzyme. In muscle and other non-liver tissues, CPT1 is associated with the outer mitochondrial membrane. CPT1 mediates the transport of long chain fatty acids across the membrane by binding them to carnitine. This enzyme can be inhibited by malonyl CoA.

The "CPT1A" form is associated with carnitine palmitoyltransferase I deficiency.