L-Carnitine
L-Carnitine is required to shuttle long-chain fatty acids across the inner mitochondrial membrane via the CPT1/carnitine-acylcarnitine-translocase/CPT2 system, the rate-limiting entry point for mitoch...
PubChem CID10917
FormulaC7H15NO3
Mol. Weight161.2 g/mol
Mechanism of Action
L-Carnitine is required to shuttle long-chain fatty acids across the inner mitochondrial membrane via the CPT1/carnitine-acylcarnitine-translocase/CPT2 system, the rate-limiting entry point for mitochondrial beta-oxidation. Genetic OCTN2 transporter defects that impair carnitine uptake cause primary carnitine deficiency, underscoring the pathway's centrality to fatty-acid-based cellular energy metabolism.
Carnitine palmitoyltransferase I/II (CPT1/CPT2)OCTN2 carnitine transporter