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Various physiological and pathological conditions can lead to an accumulation of misfolded proteins in the ER lumena conserved adaptive reaction known as ER stress, and triggering the unfolded protein response (UPR) (Liu et al., 2017)

doi: 10.1042/CS20190171 164 WangM.PanW.XuY.ZhangJ.WanJ.JiangH

Firstly, it replenishes intracellular glutathione levels, which helps protect cells from oxidative stress and damage caused by free radicals

Hohenfellner K, Bergmann C, Fleige T, Janzen N, Burggraf S, Olgemoller B, Gahl WA, Czibere L, Froschauer S, Roschinger W, Vill K, Harms E, Nennstiel U (2019) Molecular based newborn screening in Germany: follow-up for cystinosis

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