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首页> 外文期刊>IBRO Reports >CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits
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CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefits

机译：CuATSM功效在ALS的SOD1小鼠模型中独立复制，而未金属化的ATSM疗法未能显示出益处

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A copper chelator known as diacetylbis(N(4)-methylthiosemicarbazonato) copper II (CuATSM), has been reported to be efficacious in multiple transgenic SOD1 models of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder affecting motor neurons. Here we report that we also observed CuATSM efficacy on disease onset and progression in a standardized litter-matched and gender-balanced efficacy study using B6SJL-SOD1G93A/1Gur mice. We also report improved survival trends with CuATSM treatment. In addition, we report a lack of efficacy by unmetallated ATSM in the same model using the same standardized study design. These results add to existing evidence supporting an efficacious role for copper delivery using chaperone molecules in mouse models of ALS. Highlights ? CuATSM administration slows disease onset and progression in high copy SOD1 mice. ? Signs of CuATSM efficacy are more pronounced in male SOD1 mice than in female SOD1 mice. ? Unmetallated ATSM administration reveals no detectable effects on disease progression in high copy SOD1 mice.
机译：铜螯合剂称为二乙酰双（N（4）-甲基硫代半碳氮杂铜）II（CuATSM），据报道在肌萎缩性侧索硬化症（ALS）的多种转基因SOD1模型中有效，后者是一种影响运动神经元的致命性神经退行性疾病。在这里，我们报告说，在使用B6SJL-SOD1G93A / 1Gur小鼠进行标准化的同窝配对和性别平衡的功效研究中，我们还观察到了CuATSM对疾病发作和进展的功效。我们还报告了CuATSM治疗改善了生存趋势。此外，我们报告了使用相同标准化研究设计的相同模型中非金属化ATSM缺乏疗效。这些结果增加了现有证据，支持在伴侣的ALS小鼠模型中使用分子伴侣分子对铜的有效递送。强调 ？在高拷贝SOD1小鼠中，CuATSM的给药可以减缓疾病的发作和进展。 ？在雄性SOD1小鼠中，CuATSM功效的迹象比雌性SOD1小鼠更为明显。 ？未金属化的ATSM给药显示高拷贝SOD1小鼠对疾病进展无可检测的影响。

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