Article
ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function.
Neuron - 18 Nov 2015
Murakami Tetsuro, Qamar Seema, Lin Julie Qiaojin, Schierle Gabriele S Kaminski, Rees Eric, Miyashita Akinori, Costa Ana R, Dodd Roger B, Chan Fiona T S, Michel Claire H, Kronenberg-Versteeg Deborah, Li Yi, Yang Seung-Pil, Wakutani Yosuke, Meadows William, Ferry Rodylyn Rose, Dong Liang, Tartaglia Gian Gaetano, Favrin Giorgio, Lin Wen-Lang, Dickson Dennis W, Zhen Mei, Ron David, Schmitt-Ulms Gerold, Fraser Paul E, Shneider Neil A, Holt Christine, Vendruscolo Michele, Kaminski Clemens F, St George-Hyslop Peter
Abstract excerpt
The mechanisms by which mutations in FUS and other RNA binding proteins cause ALS and FTD remain controversial. We propose a model in which low-complexity (LC) domains of FUS drive its physiologically reversible assembly into membrane-free, liquid droplet and hydrogel-like structures. ALS/FTD mutations in LC or non-LC domains induce further phase transition into poorly soluble fibrillar hydrogels distinct from...
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