Carnitine palmitoyltransferase I

From Ganfyd

Jump to: navigation, search

The carnitine palmitoyltransferase I (CPT1) isoenzymes are:

  1. Carnitine palmitoyltransferase IA (CPT1A) coded for at 11q13 and expressed primarily in liver (and fibroblasts).
  2. Carnitine palmitoyltransferase IB (CPT1B) coded for at 22q13.3 and expressed in muscle

and are a key enzyme located in the outer mitochondrial membrane involved in the carnitine shuttle for long chain fatty acids.

The CPT1A gene is known to be 20 exons with several associated promoter regions and and transcription factor-binding sites. The CPT1A protein is inhibited by malonyl-CoA. It is known that promoting hypothalamic CPT1 activity in animal models diminishs food intake and endogenous glucose production.

Primary carnitine palmitoyltransferase I deficiency is a serious condition presenting in neonates and early life.

Inhibitors

Which may have potential in reducing tissue ischaemic damage by analogy include: