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In the normal physiological state (Figure 3a), GSH is produced by the condensation of the amino acids glutamate and cysteine to form the dipeptide -glutamylcysteine, which is catalyzed by the enzyme, -glutamyl cysteine synthetase.The dipeptide then condenses with the amino acid glycine to form the tripeptide GSH which is catalyzed by the enzyme GSH synthetase

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The most clinically significant of these are COL3A1 mutations causing the potentially lethal vascular form of EDS

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As for dietary factors, a lower intake of some antioxidant nutrients, such as vitamins A, C and E, carnitine, folate, zinc or selenium, has been associated with male infertility