ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function
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- 作者：Tetsuro Murakami1 ; 12 ; Seema Qamar2 ; 12 ; Julie Qiaojin Lin3 ; 12 ; Gabriele S. Kaminski Schierle4 ; 12 ; Eric Rees4 ; Akinori Miyashita1 ; Ana R. Costa2 ; Roger B. Dodd2 ; Fiona T.S. Chan4 ; Claire H. Michel4 ; Deborah Kronenberg-Versteeg2 ; Yi Li2 ; Seung-Pil Yang1 ; Yosuke Wakutani1 ; William Meadows2 ; Rodylyn Rose Ferry1 ; Liang Dong2 ; Gian Gaetano Tartaglia5 ; 6 ; Giorgio Favrin7 ; Wen-Lang Lin8 ; Dennis W. Dickson8 ; Mei Zhen9 ; David Ron10 ; Gerold Schmitt-Ulms1 ; Paul E. Fraser1 ; Neil A. Shneider11 ; Christine Holt3 ; Michele Vendruscolo5 ; Clemens F. Kaminski4 ; Peter St George-Hyslop1 ; 2 ; phs22@cam.ac.uk" class="auth_mail" title="E-mail the corresponding author ; p.hyslop@utoronto.ca" class="auth_mail" title="E-mail the corresponding author
- 刊名：Neuron
- 出版年：2015
- 出版时间：18 November 2015
- 年：2015
- 卷：88
- 期：4
- 页码：678-690
- 全文大小：3321 K
文摘
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FUS phase transitions between monomer, liquid droplet, and hydrogel states
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FUS mutants induce further phase transition into irreversible fibrillar hydrogels
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Irreversible hydrogels sequester RNP cargo and impair RNP granule function
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Formation of non-amyloid fibrillar hydrogels provides a compelling causative mechanism for neurodegeneration