Target intelligence / Profile preview

Carnitine O-palmitoyltransferase system (CPT system) (CPT system)

Target
CPT system
Molecular classification
Enzyme, Acyltransferase
01

Overview

The carnitine O-palmitoyltransferase (CPT) system is a multi-component enzyme complex essential for the mitochondrial beta-oxidation of long-chain fatty acids (StatPearls, PMID: 30020613). It comprises carnitine palmitoyltransferase 1 (CPT1), located on the outer mitochondrial membrane, and carnitine palmitoyltransferase 2 (CPT2), situated on the inner mitochondrial membrane, working in conjunction with the carnitine-acylcarnitine translocase (CACT) (UniProt P50416, P23786). CPT1 serves as the rate-limiting step of the pathway, converting long-chain acyl-CoAs into acylcarnitines for transport into the mitochondrial matrix (StatPearls, PMID: 30020613). This system is a key regulator of metabolic flexibility, allowing cells to switch between lipid and carbohydrate fuels based on energy availability and hormonal signals (PMID: 21834967). Pathological alterations in the CPT system are linked to metabolic disorders such as type 2 diabetes and obesity, as well as the metabolic reprogramming observed in many cancers (PMID: 31507451). Therapeutic strategies often target CPT1 with inhibitors like etomoxir or perhexiline to reduce fatty acid oxidation, which can improve insulin sensitivity or deprive cancer cells of a critical energy source (PubChem CID 123628, 4743).

Other names
Carnitine palmitoyltransferase complexCPT1/CPT2 systemCarnitine shuttleCarnitine acyltransferase system
02

Mechanism of action

Inhibition of CPT1 to reduce mitochondrial fatty acid uptake and promote glucose oxidation.

03

Biological functions

Fatty acid beta-oxidationLipid metabolismMitochondrial transportEnergy homeostasis
04

Disease associations

Type 2 diabetesHeart failureCancerObesityIschemic heart diseaseCarnitine palmitoyltransferase deficiency
05

Safety considerations

HepatotoxicityCardiac hypertrophyMyopathySteatosis
06

Interacting drugs

Etomoxir

4 more in the full profile.

07

Biomarkers

Plasma acylcarnitine levelsMalonyl-CoA levelsCPT1A expressionCPT1B expression

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