Carnitine palmitoyltransferase I (CPT-1)
Cofactors used
Clinical / pharmacological. CPT-I deficiency: hypoketotic hypoglycaemia, hepatomegaly
What it does, reaction by reaction
Beta-Oxidation of Fatty Acids Lipid Metabolism · Mitochondrial matrix (peroxisome for VLCFA)
Fatty acyl-CoA + carnitine → acylcarnitine + CoA-SH
Converts Fatty acyl-CoA Carnitine into Acylcarnitine CoA-SH
› Notes
Carnitine palmitoyltransferase I (CPT-1), located on the outer mitochondrial membrane, transfers the acyl group to carnitine. This is the principal rate-limiting/regulatory step for mitochondrial oxidation of long-chain fatty acids and is inhibited by malonyl-CoA.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
No regulation recorded for this enzyme in the source documents.
Recent literature
Nothing is fetched until you ask, so the page stays fast and the request is yours rather than automatic.
Europe PMC · ten most recent, newest first