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Shaping the role of mitochondria in the pathogenesis of Huntington's disease

机译:线粒体的塑造作用亨廷顿氏病的发病机理

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摘要

Intense research on the pathogenesis of Huntington's disease (HD), a genetic neurodegenerative disease caused by a polyglutamine expansion in the Huntingtin (Htt) protein, revealed multiple potential mechanisms, among which mitochondrial alterations had emerged as key determinants of the natural history of the disease. Pharmacological and genetic animal models of mitochondrial dysfunction in the striatum, which is mostly affected in HD corroborated a key role for these organelles in the pathogenesis of the disease. Here, we will give an account of the recent evidence indicating that the mitochondria-shaping machinery is altered in HD models and patients. Since its correction can counteract HD mitochondrial dysfunction and cellular damage, drugs impacting on mitochondrial shape are emerging as a new possibility of treatment for this devastating condition.
机译:强烈的发病机理的研究亨廷顿氏病(HD),一个基因神经退行性疾病引起的polyglutamine扩张杭丁顿蛋白(计画)蛋白质,显示多个潜在的机制,其中线粒体的改变就出现了的自然历史的关键因素疾病。模型的线粒体功能障碍纹状体,主要是影响高清证实这些细胞器的关键作用疾病的发病机理。最近的证据表明的叙述mitochondria-shaping机械改变在HD模型和病人。修正可以抵消高清线粒体功能障碍和细胞损伤、药物影响在线粒体正在成为一个新的形状治疗这种毁灭性的可能性条件。

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