The DOI matches its Crossref record; 40 of the 40 references it cites were found in a registry or an index.
Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains
Open accessAbstract
Summary 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 fibrillization and cause disease. Here, we establish that nuclear-import receptors (NIRs) specifically chaperone and potently disaggregate wild-type and disease-linked RBPs bearing a NLS. Karyopherin-β2 (also called Transportin-1) engages PY-NLSs to inhibit and reverse FUS, TAF15, EWSR1, hnRNPA1, and hnRNPA2 fibrillization, whereas Importin-α plus Karyopherin-β1 prevent and reverse TDP-43 fibrillization. Remarkably, Karyopherin-β2 dissolves phase-separated liquids and aberrant fibrillar hydrogels formed by FUS and hnRNPA1. In vivo, Karyopherin-β2 prevents RBPs with PY-NLSs accumulating in stress granules, restores nuclear RBP localization and function, and rescues degeneration caused by disease-linked FUS and hnRNPA2. Thus, NIRs therapeutically restore RBP homeostasis and mitigate neurodegeneration.
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Self-citationMutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
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Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis
Prasad, A., Bharathi, V., Sivalingam, V., Girdhar, A., & Patel, B. K. (2019) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2019.00025 · Cited by 774
Metadata
- Authors
- Guo, L., Kim, H. J., Wang, H., Monaghan, J., Freyermuth, F., Sung, J. C., O’Donovan, K., Fare, C. M., Diaz, Z., Singh, N., Zhang, Z. C., Coughlin, M., Sweeny, E. A., DeSantis, M. E., Jackrel, M. E., Rodell, C. B., Burdick, J. A., King, O. D., Gitler, A. D., Lagier-Tourenne, C., Pandey, U. B., Chook, Y. M., Taylor, J. P., & Shorter, J.
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 2018-04
- Volume
- 173
- Issue
- 3
- Pages
- 677-692.e20
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2802842075
- Cited by
- 466
- References
- 40
- FWCI
- 33.94
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Guo, L., Kim, H. J., Wang, H., Monaghan, J., Freyermuth, F., Sung, J. C., O’Donovan, K., Fare, C. M., Diaz, Z., Singh, N., Zhang, Z. C., Coughlin, M., Sweeny, E. A., DeSantis, M. E., Jackrel, M. E., Rodell, C. B., Burdick, J. A., King, O. D., Gitler, A. D., . . . Shorter, J. (2018). Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains. Cell. https://doi.org/10.1016/j.cell.2018.03.002
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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