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| Huntington’s disease (HD) is a devastating neurodegenerative disorder characterized by progressive motor dysfunction, dementia and emotional disturbances. It is inherited in an autosomal dominant manner and its prevalence is 5–10cases per 100,000.The HD gene codes for a large highly conserved protein named huntingtin. In affected individuals, there is an expanded polyglutamine sequence in the protein owing to the expansion of a trinucleotide repeat sequence (CAGn) near the 50 end of the gene. An inverse relationship exists between the CAG repeat number (i.e. glutamine residues) and the age of onset of the first symptoms, with higher repeat numbers associated with a younger age of onset. Mutant huntingtin forms aggregates with itself and other proteins and it is believed to confer toxicity via a gain-of-function mechanism [124]. When the huntingtin aggregates this phenomenon induces a gradual atrophy of the striatum. Hence, one possible way to slow or attenuate the effects of the HD mutation may be to enhance the removal of the mutant protein, because it acts as a toxin. Since the autophagy-lysosomal pathway is a major route for protein clearance in eukaryotic cells, it has been demonstrated that also mutant huntingtin requests to be cleared by (macro)autophagy [124]. The autophagic pathway involves the formation of double membrane structures around a portion of cytosol, which then fuse with lysosomes where their contents are degraded. In a mouse model of HD, the only one way to induce autophagy has been to inhibit the protein kinase mTOR with the molecule CCI-779, a rapamycin analogue [124]. This inhibition has improved behavioural performance and decreased aggregate formation in this mouse model. However, because the kinase mTOR pathway controls several cellular processes besides autophagy, blocking its activity probably contributes to the complications seen with long-term use of its inhibitor [124]. Therefore, promoting clearance of mutant huntingtin (mhtt) by induction of macroautophagy can be considered a valid approach for treating human HD [124]. |
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| 亨廷顿舞蹈病（HD） 是一种以渐进的运动功能障碍、 痴呆及情绪障碍为特点的破坏性神经退行性疾病。它继承的常染色体显性遗传为主导方式，其发生率是每一名为 HD的大型高保守蛋白 100,000。这种HD 基因代码为5–10cases。在受影响的个人中，由于三核苷酸重复序列 （CAGn） 附近的基因 50 结束扩张，蛋白质有扩大的 polyglutamine 序列。CAG 重复编号之间 (即成反比关系，并有谷氨酰胺残留) 和焦虑症状的出现，这与发病年龄相关。通过增益函数机制 [124] ，我们相信突变体 以huntingtin 形式本身和其他蛋白质相互作用。当Huntingtin 聚合时，这种现象诱导纹状体的逐步萎缩。因此，一个可能的途径或衰减 HD 突变的影响就可能是减少突变蛋白质，因为它是一种毒素。由于自噬溶酶体通路是真核细胞中蛋白清除的重要途径，科学已经证明这也是突变体 huntingtin 请求被清除的自噬 [124]。 自噬的通路涉及双膜结构周围的部分细胞，然后在其内与已降级的溶酶体融合。其中房屋署的小鼠模型，诱使自噬时只有一种方法：一直抑制蛋白激酶 mTOR 分子与雷帕霉素 CCI-779 [124]。 这抑制了改进的行为表现和减少其在此模型中的聚合形成。但是，因为激酶 mTOR 通路控制几个细胞的过程，除了自噬，还可以阻止其活动，这可能有助于其抑制剂长期使用的并发症[124]。 因此，促进突变体 huntingtin 的间隙 (mhtt) macroautophagy 感应可被视为一种有效的治疗人类 HD 方法[124]。 |
2楼2012-04-17 13:06:12
