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Together these results indicate that the succination of GSH, but not its detoxification, is responsible for the oxidative stress observed in Fh1-deficient cells GSH succination increases GSH biosynthesis We hypothesized that the chronic depletion of GSH caused by succination could perturb cell metabolism and contribute to a general deregulation of redox homeostasis

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doi: 10.1056/NEJM199402033300508

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