Hsp70 chaperones TDP-43 in dynamic, liquid-like phase and prevents it from amyloid aggregation.
作者信息
Gu Jinge, Wang Chen, Hu Rirong, Li Yichen, Zhang Shengnan, Sun Yunpeng, Wang Qiangqiang, Li Dan, Fang Yanshan, Liu Cong
机构信息
Interdisciplinary Research Center on Biology and Chemistry, Shanghai Institute of Organic Chemistry, Chinese Academy of Sciences, Shanghai, China.
University of Chinese Academy of Sciences, Beijing, China.
出版信息
Cell Res. 2021 Sep;31(9):1024-1027. doi: 10.1038/s41422-021-00526-5. Epub 2021 Jul 8.
Abstract
摘要
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HSP70 chaperones RNA-free TDP-43 into anisotropic intranuclear liquid spherical shells.热休克蛋白 70 伴侣将无 RNA 的 TDP-43 成核内各向异性液滴状球形壳。
Science. 2021 Feb 5;371(6529). doi: 10.1126/science.abb4309. Epub 2020 Dec 17.
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Stress Induces Dynamic, Cytotoxicity-Antagonizing TDP-43 Nuclear Bodies via Paraspeckle LncRNA NEAT1-Mediated Liquid-Liquid Phase Separation.应激通过核内包涵体特异性细丝相关蛋白 NEAT1 介导的液-液相分离诱导动态、细胞毒性拮抗 TDP-43 核体。
Mol Cell. 2020 Aug 6;79(3):443-458.e7. doi: 10.1016/j.molcel.2020.06.019. Epub 2020 Jul 9.
3
TDP-43 α-helical structure tunes liquid-liquid phase separation and function.TDP-43 的 α-螺旋结构调节液-液相分离和功能。
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RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy.RRM 临近 TARDBP 突变破坏 RNA 结合并增强 TDP-43 蛋白病。
Brain. 2019 Dec 1;142(12):3753-3770. doi: 10.1093/brain/awz313.
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Cell Rep. 2017 Oct 3;21(1):110-125. doi: 10.1016/j.celrep.2017.09.028.
6
ALS Mutations Disrupt Phase Separation Mediated by α-Helical Structure in the TDP-43 Low-Complexity C-Terminal Domain.肌萎缩侧索硬化症突变破坏了由TDP - 43低复杂性C末端结构域中的α - 螺旋结构介导的相分离。
Structure. 2016 Sep 6;24(9):1537-49. doi: 10.1016/j.str.2016.07.007. Epub 2016 Aug 18.
7
The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis.热休克反应在TDP-43清除中起重要作用：肌萎缩侧索硬化症功能障碍的证据。
Brain. 2016 May;139(Pt 5):1417-32. doi: 10.1093/brain/aww028. Epub 2016 Mar 1.
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Altered ribostasis: RNA-protein granules in degenerative disorders.核糖休改变：变性疾病中的 RNA-蛋白质颗粒。
Cell. 2013 Aug 15;154(4):727-36. doi: 10.1016/j.cell.2013.07.038.
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Wild-type and A315T mutant TDP-43 exert differential neurotoxicity in a Drosophila model of ALS.野生型和 A315T 突变 TDP-43 在 ALS 的果蝇模型中表现出不同的神经毒性。
Hum Mol Genet. 2011 Jun 15;20(12):2308-21. doi: 10.1093/hmg/ddr124. Epub 2011 Mar 26.
10
TAR DNA-binding protein 43 in neurodegenerative disease.TAR DNA 结合蛋白 43 在神经退行性疾病中的作用。
Nat Rev Neurol. 2010 Apr;6(4):211-20. doi: 10.1038/nrneurol.2010.18. Epub 2010 Mar 16.
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