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The synthesis of GSH from its constituent amino acids involves two ATP-requiring enzymatic steps: L-glutamate + L-cysteine + ATP -glutamyl-L-cysteine + ADP + Pi -glutamyl-L-cysteine + L-glycine + ATP GSH + ADP + Pi The first step of GSH biosynthesis is considered rate limiting and catalyzed by glutamate cysteine ligase (GCL, EC 6.3.2.2

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By enhancing the active transport of long-chain fatty acids into the mitochondrial matrix via the carnitine shuttle, L-carnitine supplementation supports robust, sustained beta-oxidation