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Dormann, Dorothee (2016): FUScinating insights into motor neuron degeneration. In: Embo Journal, Vol. 35, No. 10: pp. 1015-1017
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Point mutations in FUS cause amyotrophic lateral sclerosis (ALS), a devastating neurodegenerative disease-but do they do that by a loss of the protein's normal function, or by endowing it with novel toxic functions, or both? In this issue of The EMBO Journal, Scekic-Zahirovic et al (2016) report that mutant FUS, but not the complete loss of FUS, triggers motor neuron degeneration in mice, arguing for a toxic gain-of-function mechanism.