l-carnitine transports fatty acids into mitochondria Carnitine shuttle system and beta oxidation of Mitochondrial protein promotes carnitine synthesis

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The γ-glutamyl cycle, comprising six enzymes, harbors four hereditary defects: γ-glutamylcysteine synthetase, glutathione synthetase, γ-glutamyl transpeptidase, and 5-oxoprolinase

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Carnitine is naturally present in many foods and most of the daily requirements of L-carnitine is provided through the diet