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In GS deficiency, glutamine deficiency results in reduced NAD + synthesis, which plays an important role in redox reactions.6 In GLS hyperactivity, glutamate excess is postulated to decrease redox buffer capacity.19 As oxidative stress is associated with cataract, neurodegenerative disorders and epilepsy, this is a likely key player in the pathophysiology of these inborn errors of glutamate metabolism.10, 20, 88 In UCDs, glutamine concentrations are increased as a consequence of hyperammonemia
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Glutathione is the most important antioxidant support nutrient produced in the body and is found in every cell