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If the cells supply of glutathione is severely depleted and GSSG trafficking out of the cell exceeds the rate at which it can be reduced back to GSH, cellular defence against oxidative stress is impaired and the cell may experience critical membrane failure and even cell death as a result of lipid peroxidation due to lack of protective glutathione antioxidant action

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Also, a mouse model for late-onset mitochondrial myopathy due to overexpression of mutated Twinkle protein, a nuclear-encoded replicative helicase of mtDNA, showed clear health improvements after 10 months of KD