ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function

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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

•: FUS phase transitions between monomer, liquid droplet, and hydrogel states
•: FUS mutants induce further phase transition into irreversible fibrillar hydrogels
•: Irreversible hydrogels sequester RNP cargo and impair RNP granule function
•: Formation of non-amyloid fibrillar hydrogels provides a compelling causative mechanism for neurodegeneration

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