Rhes deletion is neuroprotective in the 3-nitropropionic acid model of Huntington's disease.
Rhes deletion is neuroprotective in the 3-nitropropionic acid model of Huntington's disease.
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DOI：
10.1523/jneurosci.3730-12.2013
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发表时间：
2013-02-27
期刊：
影响因子：
--
通讯作者：
Snyder SH
中科院分区：
文献类型：
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作者：
Mealer RG;Subramaniam S;Snyder SH
虽然引起亨廷顿病（HD）的突变蛋白在全身表达，但HD的主要病理定位于脑的纹状体。我们以前报道，纹状体富集蛋白Rhes结合突变的亨廷顿蛋白，并增强其细胞毒性。我们现在证明，在由3-硝基丙酸引起的纹状体特异性HD模型中，Rhes缺失的小鼠被显着保护免受神经毒性和运动功能障碍。这一发现表明，Rhes可能，在一定程度上，决定了纹状体的选择性HD。
Although the mutated protein causing Huntington's disease (HD) is expressed throughout the body, the major pathology of HD is localized to the striatum of the brain. We previously reported that the striatal-enriched protein Rhes binds the mutated huntingtin protein and enhances its cytotoxicity. We now demonstrate that Rhes-deleted mice are dramatically protected from neurotoxicity and motor dysfunction in a striatal-specific model of HD elicited by 3-nitropropionic acid. This finding suggests that Rhes may, in part, determine the striatal selectivity of HD.