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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
HbA1c reductions, improved insulin sensitivity, and potentially reduced need for diabetes medications can be transformative health outcomes that the scale does not capture
These health complications not only diminish quality of life but also impose substantial economic burdens on healthcare systems globally
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