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Neurodegeneration

Problems at the nuclear pore

Nature volume 525, pages 36–37 (2015)Cite this article

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Expansion of a repetitive DNA sequence is associated with neurodegeneration. Three studies identify genes involved in nuclear import and export that can mediate the toxicity this expansion causes. See Article p.56 & Letter p.129

Advances in molecular genetics and stem-cell technology are transforming our understanding of disease. Such progress is desperately needed in amyotrophic lateral sclerosis, a paralysing neurodegenerative disease that is almost uniformly fatal. It is therefore welcome news that three studies (two in this issue1,2 and one in Nature Neuroscience3) have converged on a molecular mechanism that seems to underlie a familial form of the disease.

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Figure 1: Models of G4C2-mediated neurotoxicity.

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Authors and Affiliations

  1. Bennett W. Fox and Randal S. Tibbetts are in the Department of Human Oncology, University of Wisconsin–Madison, Madison, Wisconsin 53706, USA.,

    Bennett W. Fox & Randal S. Tibbetts

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  1. Bennett W. Fox
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  2. Randal S. Tibbetts
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Correspondence to Randal S. Tibbetts.

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Fox, B., Tibbetts, R. Problems at the nuclear pore. Nature 525, 36–37 (2015). https://doi.org/10.1038/nature15208

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