Article
Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains.
Cell - 19 Apr 2018
Guo Lin, Kim Hong Joo, Wang Hejia, Monaghan John, Freyermuth Fernande, Sung Julie C, O'Donovan Kevin, Fare Charlotte M, Diaz Zamia, Singh Nikita, Zhang Zi Chao, Coughlin Maura, Sweeny Elizabeth A, DeSantis Morgan E, Jackrel Meredith E, Rodell Christopher B, Burdick Jason A, King Oliver D, Gitler Aaron D, Lagier-Tourenne Clotilde, Pandey Udai Bhan, Chook Yuh Min, Taylor J Paul, Shorter James
Abstract excerpt
RNA-binding proteins (RBPs) with prion-like domains (PrLDs) phase transition to functional liquids, which can mature into aberrant hydrogels composed of pathological fibrils that underpin fatal neurodegenerative disorders. Several nuclear RBPs with PrLDs, including TDP-43, FUS, hnRNPA1, and hnRNPA2, mislocalize to cytoplasmic inclusions in neurodegenerative disorders, and mutations in their PrLDs can accelerate...
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