Rhes 缺失对 3-硝基丙酸诱导的亨廷顿病模型具有神经保护作用。
Rhes deletion is neuroprotective in the 3-nitropropionic acid model of Huntington's disease.
机构信息
Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
出版信息
J Neurosci. 2013 Feb 27;33(9):4206-10. doi: 10.1523/JNEUROSCI.3730-12.2013.
Abstract
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.
摘要
尽管导致亨廷顿病（HD）的突变蛋白在全身表达，但 HD 的主要病理学位于大脑的纹状体。我们之前报道过，富含纹状体的 Rhes 蛋白与突变的亨廷顿蛋白结合并增强其细胞毒性。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 缺失小鼠对神经毒性和运动功能障碍有明显的保护作用。这一发现表明，Rhes 可能部分决定了 HD 的纹状体选择性。
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