Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains

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,...

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Veröffentlicht in:	Cell 2018-04, Vol.173 (3), p.677-692.e20
Hauptverfasser:	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
Format:	Artikel
Sprache:	eng
Schlagworte:	Active Transport, Cell Nucleus Adult Aged ALS Animals beta Karyopherins - chemistry Cytoplasm - chemistry cytoplasmic granules cytoplasmic inclusions disaggregase DNA-Binding Proteins - chemistry Drosophila melanogaster Female FTD FUS Green Fluorescent Proteins - chemistry HEK293 Cells HeLa Cells hnRNPA1 Homeostasis Humans hydrogels importins Karyopherin-β2 Karyopherins - chemistry Male Middle Aged Molecular Chaperones - chemistry Mutation neurodegeneration neurodegenerative diseases Neurodegenerative Diseases - pathology Nuclear-important receptor phase transition physiological transport Prions - chemistry Protein Domains receptors Receptors, Cytoplasmic and Nuclear - chemistry RNA-Binding Protein EWS - chemistry RNA-binding proteins RNA-Binding Proteins - chemistry TATA-Binding Protein Associated Factors - chemistry TDP-43
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