Suppr超能文献

雷帕霉素靶蛋白复合体 2 水平升高可预防突变型亨廷顿蛋白诱导的神经元变性。

Increased Levels of Rictor Prevent Mutant Huntingtin-Induced Neuronal Degeneration.

机构信息

Departament de Biomedicina, Facultat de Medicina i Ciències de la Salut, Institut de Neurociències, Universitat de Barcelona, Casanova 143, E-08036, Barcelona, Catalonia, Spain.

Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain.

出版信息

Mol Neurobiol. 2018 Oct;55(10):7728-7742. doi: 10.1007/s12035-018-0956-5. Epub 2018 Feb 19.

Abstract

Rictor associates with mTOR to form the mTORC2 complex, which activity regulates neuronal function and survival. Neurodegenerative diseases are characterized by the presence of neuronal dysfunction and cell death in specific brain regions such as for example Huntington's disease (HD), which is characterized by the loss of striatal projection neurons leading to motor dysfunction. Although HD is caused by the expression of mutant huntingtin, cell death occurs gradually suggesting that neurons have the capability to activate compensatory mechanisms to deal with neuronal dysfunction and later cell death. Here, we analyzed whether mTORC2 activity could be altered by the presence of mutant huntingtin. We observed that Rictor levels are specifically increased in the striatum of HD mouse models and in the putamen of HD patients. Rictor-mTOR interaction and the phosphorylation levels of Akt, one of the targets of the mTORC2 complex, were increased in the striatum of the R6/1 mouse model of HD suggesting increased mTORC2 signaling. Interestingly, acute downregulation of Rictor in striatal cells in vitro reduced mTORC2 activity, as shown by reduced levels of phospho-Akt, and increased mutant huntingtin-induced cell death. Accordingly, overexpression of Rictor increased mTORC2 activity counteracting cell death. Furthermore, normalization of endogenous Rictor levels in the striatum of R6/1 mouse worsened motor symptoms suggesting an induction of neuronal dysfunction. In conclusion, our results suggest that increased Rictor striatal levels could counteract neuronal dysfunction induced by mutant huntingtin.

摘要

Rictor 与 mTOR 结合形成 mTORC2 复合物,其活性调节神经元功能和存活。神经退行性疾病的特征是特定脑区(例如亨廷顿病,HD)存在神经元功能障碍和细胞死亡,HD 表现为纹状体投射神经元的丧失导致运动功能障碍。尽管 HD 是由突变亨廷顿蛋白的表达引起的,但细胞死亡是逐渐发生的,这表明神经元有能力激活代偿机制来应对神经元功能障碍和随后的细胞死亡。在这里,我们分析了突变 huntingtin 的存在是否会改变 mTORC2 的活性。我们观察到 Rictor 水平在 HD 小鼠模型的纹状体和 HD 患者的壳核中特异性增加。Rictor-mTOR 相互作用和 Akt 的磷酸化水平(mTORC2 复合物的靶标之一)在 HD 的 R6/1 小鼠模型的纹状体中增加,表明 mTORC2 信号增加。有趣的是,体外急性下调纹状体细胞中的 Rictor 降低了 mTORC2 活性,如磷酸化 Akt 水平降低所示,并增加了突变 huntingtin 诱导的细胞死亡。相应地,Rictor 的过表达增加了 mTORC2 活性,抵消了细胞死亡。此外,R6/1 小鼠纹状体中内源性 Rictor 水平的正常化恶化了运动症状,表明神经元功能障碍的诱导。总之,我们的结果表明纹状体中 Rictor 水平的增加可能抵消突变 huntingtin 诱导的神经元功能障碍。

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验