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However, in glutathione synthetase deficiency, because the activity of gamma-glutamyl-cysteine synthetase is regulated via feedback inhibition by glutathione, gamma-glutamyl-cysteine and then L-5-oxoproline are overproduced, resulting in the metabolic acidosis and urinary excretion of this compound at levels several hundred-fold above normal (71

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Size:3mL* 10 vials

These changes in intestinal-level metabolic processes due to lack of gut microbes are reflected in the altered extra-intestinal metabolism and neurological phenotype 12,13