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Title:

Phase Separation of FUS Is Suppressed by Its Nuclear Import Receptor and Arginine Methylation.

Document type:
Journal Article; Article
Author(s):
Hofweber, Mario; Hutten, Saskia; Bourgeois, Benjamin; Spreitzer, Emil; Niedner-Boblenz, Annika; Schifferer, Martina; Ruepp, Marc-David; Simons, Mikael; Niessing, Dierk; Madl, Tobias; Dormann, Dorothee
Abstract:
Cytoplasmic FUS aggregates are a pathological hallmark in a subset of patients with frontotemporal dementia (FTD) or amyotrophic lateral sclerosis (ALS). A key step that is disrupted in these patients is nuclear import of FUS mediated by the import receptor Transportin/Karyopherin-?2. In ALS-FUS patients, this is caused by mutations in the nuclear localization signal (NLS) of FUS that weaken Transportin binding. In FTD-FUS patients, Transportin is aggregated, and post-translational arginine meth...     »
Journal title abbreviation:
Cell
Year:
2018
Journal volume:
173
Journal issue:
3
Pages contribution:
706-719.e13
Fulltext / DOI:
doi:10.1016/j.cell.2018.03.004
Pubmed ID:
http://view.ncbi.nlm.nih.gov/pubmed/29677514
Print-ISSN:
0092-8674
TUM Institution:
Lehrstuhl für Zellbiologie des Nervensystems (Prof. Misgeld)
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