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Lipoic acid improves survival in transgenic mouse models of Huntington's disease.

机译:硫辛酸可改善亨廷顿氏病转基因小鼠模型的存活率。

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摘要

There is substantial evidence implicating excitotoxicity and oxidative damage in the pathogenesis of Huntington's disease (HD). We therefore examined whether the antioxidants 2-sulpho-tert-phenyibutyinitrone (S-PBN) and alpha-lipoic acid could exert neuroprotective effects in transgenic mouse models of HD. S-PBN showed no effects on either weight loss or survival in the R6/2 transgenic HD mice. alpha-Lipoic acid produced significant increases in survival in both R6/2 and N171-82Q transgenic mouse models of HD. These findings suggest that alpha-lipoic acid might have beneficial effects in HD patients.
机译:有大量证据表明在亨廷顿舞蹈病(HD)的发病机理中存在兴奋性毒性和氧化损伤。因此,我们检查了抗氧化剂2-硫代-叔丁基苯丁酮(S-PBN)和α-硫辛酸能否在HD转基因小鼠模型中发挥神经保护作用。 S-PBN对R6 / 2转基因HD小鼠的体重减轻或生存均无影响。在HD的R6 / 2和N171-82Q转基因小鼠模型中,α-硫辛酸均可显着提高存活率。这些发现表明,α-硫辛酸可能对HD患者具有有益的作用。

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