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Machado‐Joseph disease (MJD)/spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disorder caused by polyglutamine expansion in the ataxin‐3 protein that confers a toxic gain of function. Because of the late onset of the disease, we hypothesize that the accumulated oxidative stress or/and defective antioxidant enzyme ability may be contributory factors in the pathogenesis of MJD. In this study, we utilized SK‐N‐SH and COS7 cells stably transfected with full‐length MJD with 78 polyglutamine repeats to examine any alterations in the antioxidant activity. We demonstrated a significant reduction in the ratio of GSH/GSSG and total glutathione content (GSH + 2× GSSG) in mutant MJD cells compared with the wild‐type cells under normal or stressful conditions. We also showed that both SK‐N‐SH‐MJD78 and COS7‐MJD78‐GFP cell lines have lower activities of catalase, glutathione …