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L-Carnitine (β-hydroxy-γ-N-trimethylaminobutyric acid, C₇H₁₅NO₃, MW 161.20) is a water-soluble zwitterionic amino acid derivative essential for energy metabolism. It is endogenously synthesized mainly in the liver and kidneys from lysine and methionine, and obtained from dietary sources like red meat and dairyPMC. Only the L-isomer is biologically active in humans; the D-isomer is inhibitory and toxic. Its primary role is to transport long-chain fatty acids across the mitochondrial membrane via the carnitine-acylcarnitine cycle (involving CPT I, CACT, CPT II) for β-oxidation, supporting cellular ATP productionPMC. It also clears toxic acyl-CoA derivatives and maintains CoA homeostasis. L-Carnitine is conditionally essential: healthy adults synthesize sufficient amounts, but deficiencies may occur in neonates, those with metabolic disorders, or strict vegetariansPMCPMC
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