Article
ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism.
Human molecular genetics - 1 Jan 2012
Murakami Tetsuro, Yang Seung-Pil, Xie Lin, Kawano Taizo, Fu Donald, Mukai Asuka, Bohm Christopher, Chen Fusheng, Robertson Janice, Suzuki Hiroshi, Tartaglia Gian Gaetano, Vendruscolo Michele, Kaminski Schierle Gabriele S, Chan Fiona T S, Moloney Aileen, Crowther Damian, Kaminski Clemens F, Zhen Mei, St George-Hyslop Peter
Abstract excerpt
It is unclear whether mutations in fused in sarcoma (FUS) cause familial amyotrophic lateral sclerosis via a loss-of-function effect due to titrating FUS from the nucleus or a gain-of-function effect from cytoplasmic overabundance. To investigate this question, we generated a series of independent Caenorhabditis elegans lines expressing mutant or wild-type (WT) human FUS. We show that mutant FUS, but not WT-FUS,...
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