. ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function. Nat Commun. 2016 Feb 4;7:10465. PubMed.


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  1. I think this is very nice work. It shows that FUS nuclear localization signal mutants may act through a toxic gain of function rather than a loss of function, which is fully consistent with the idea that aberrant phase transitions are the underlying mechanism. The defect seems to be qualitative rather than quantitative, which could be because of the specific recruitment of essential factors into aberrant granules that contain mutant FUS. Whether there really is no cross-seeding with wild-type FUS is difficult to tell from the mostly genetic data that are presented. The study points to perturbation of neuronal transport granules or local translation as the primary cause of the disease. These mice will be very helpful in figuring out the molecular mechanism and the cell biology underlying ALS.

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