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Target intelligence / Profile preview
Carnitine palmitoyltransferase 1 (CPT1)
- Target
- CPT1
- Molecular classification
- Enzyme, Transferase, Carnitine acyltransferase
01
Overview
Carnitine palmitoyltransferase 1 (CPT1) is a mitochondrial enzyme critical for the metabolism of long-chain fatty acids. It catalyzes the transfer of an acyl group from long-chain fatty acyl-CoA to L-carnitine, forming acylcarnitines. This reaction is essential for transporting fatty acids from the cytosol into mitochondria, where they undergo β-oxidation to produce energy. There are three known isoforms: CPT1A (liver), CPT1B (muscle), and CPT1C (brain). CPT1 activity is rate-limiting in mitochondrial fatty acid oxidation and is tightly regulated by malonyl-CoA.
- Other names
- CPT ICAT ICCATPalmitoylCoA transferase I
02
Mechanism of action
Malonyl-CoA inhibits CPT1 activity
03
Biological functions
Lipid metabolismFatty acid transportMitochondrial fatty acid oxidationEnergy homeostasis
04
Disease associations
Hypoketotic hypoglycemiaHepatic encephalopathyMetabolic disorders
05
Safety considerations
Hypoketotic hypoglycemia during fasting or illnessRisk of hepatic encephalopathy due to toxin buildupPotential nervous system damage, liver failure, coma, or sudden death if untreated
06
Biomarkers
Elevated carnitine levels
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