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Pharmacological inhibition of GPX4 accelerates motor neuron degeneration and death (366, 367, 369), whereas GPX4 overexpression delays disease onset, improves motor function, and prolongs survival (368, 371)

10.1023/A:1005592401947 5

In this synthesis process, cysteine (Cys) is the limiting substrate and its cellular uptake as the oxidized form, cystine (Cyss), is mediated by the cystine/glutamate antiporter xCT (encoded by the SLC7A11 gene)

However, the role of thioredoxins in the protection against oxidative stress in these subjects is not clear and warrants further study

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