Article
Phase Separation of FUS Is Suppressed by Its Nuclear Import Receptor and Arginine Methylation.
Cell - 19 Apr 2018
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 excerpt
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-β2. In ALS-FUS patients, this is caused by mutations in the nuclear localization signal (NLS) of FUS that weaken Transportin binding....
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