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Tag: gamma-glutamyl cycle

Inborn errors in the metabolism of glutathione

This review discusses rare inherited disorders affecting the metabolism of glutathione, a key antioxidant involved in detoxification, redox regulation, and cellular protection. These inborn errors occur in five of the six enzymes of the gamma-glutamyl cycle and include glutathione synthetase deficiency (the most common), gamma-glutamylcysteine synthetase deficiency, gamma-glutamyl transpeptidase deficiency,

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June 26, 2025 No Comments

Physiological and pathological aspects of GSH metabolism

This review article examines the physiological and pathological roles of glutathione (GSH), focusing on its essential antioxidant function and its involvement in diseases related to oxidative stress, especially in neonates. It details the gamma-glutamyl cycle, the key enzymes responsible for GSH synthesis and recycling, and the severe consequences of enzyme

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June 26, 2025 No Comments