Pacheco Laura Segabinazzi, da Silveira Aron Ferreira, Trott Alexis, Houenou Lucien J, Algarve Thaís Doeler, Belló Caroline, Lenz Adriano Flesch, Mânica-Cattani Maria Fernanda, da Cruz Ivana Beatrice Mânica
Center for Health Sciences, Federal University of Santa Maria, UFSM, Brazil.
Mutat Res Genet Toxicol Environ Mutagen. 2013 Oct 9;757(2):99-103. doi: 10.1016/j.mrgentox.2013.06.023. Epub 2013 Aug 30.
Spinocerebellar ataxia type 3, also called Machado-Joseph disease (MJD), is an hereditary autosomal dominant neurodegenerative disease that affects the cerebellum and its afferent and efferent connections. Since the mechanism by which mutant ataxin-3 eventually leads to neuronal death is poorly understood, additional investigations to clarify the biological alterations related to Machado-Joseph disease are necessary. Recent investigations suggest that oxidative stress may contribute significantly to Machado-Joseph disease. We compared markers of oxidative stress between Machado-Joseph disease and healthy control subjects. The results showed that Machado-Joseph patients have higher catalase levels and lower thiol protein levels compared to control subjects. The peripheral blood lymphocyes of MJD patients also showed higher levels of DNA damage by the comet assay than control subjects. Our results corroborate the hypothesis that the oxidative stress is associated with MJD patients. However, whether strategies to increase cellular antioxidative capacity may be effective therapies for the treatment of Machado-Joseph disease is an open question.
3型脊髓小脑共济失调，也称为马查多-约瑟夫病（MJD），是一种遗传性常染色体显性神经退行性疾病，会影响小脑及其传入和传出连接。由于突变型ataxin-3最终导致神经元死亡的机制尚不清楚，因此有必要进行更多研究以阐明与马查多-约瑟夫病相关的生物学改变。最近的研究表明，氧化应激可能在很大程度上导致马查多-约瑟夫病。我们比较了马查多-约瑟夫病患者和健康对照者的氧化应激标志物。结果显示，与对照者相比，马查多-约瑟夫病患者的过氧化氢酶水平较高，硫醇蛋白水平较低。通过彗星试验检测，MJD患者外周血淋巴细胞的DNA损伤水平也高于对照者。我们的结果证实了氧化应激与MJD患者相关的假说。然而，提高细胞抗氧化能力的策略是否可能成为治疗马查多-约瑟夫病的有效疗法仍是一个悬而未决的问题。
