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Specifically, TfR1, GPX4, SLC7A11, SLC3A2 and SLC40A1 were significantly downregulated, while FTH/L, ACSL4, HO-1, NCOA4 and LC3II were upregulated (Fig

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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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The samples were centrifuged, and the supernatant was subjected to a GSH assay based on a kinetic enzymatic recycling method that detects the oxidation of GSH by 5,5-dithiobis-2-nitrobenzoic acid (DTNB) and glutathione reductase to measure the GSH content in cells 74