| Autor: |
Kevin J. Verstrepen, Philip Van Damme, Wim Robberecht, Ana Jovičić, Elke Bogaert, Greet De Baets, Anne Sieben, Ilse Gijselinck, Wendy Scheveneels, Emiel Michiels, Aaron D. Gitler, Marc Cruts, Ivy Cuijt, Frederic Rousseau, Christine Van Broeckhoven, Jolien Steyaert, Ludo Van Den Bosch, Steven Boeynaems, Patrick Callaerts, Joost Schymkowitz |
| Jazyk: |
angličtina |
| Rok vydání: |
2016 |
| Předmět: |
|
| Zdroj: |
Scientific Reports
Scientific reports
SCIENTIFIC REPORTS |
| ISSN: |
2045-2322 |
| Popis: |
Hexanucleotide repeat expansions in C9orf72 are the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal degeneration (FTD) (c9ALS/FTD). Unconventional translation of these repeats produces dipeptide repeat proteins (DPRs) that may cause neurodegeneration. We performed a modifier screen in Drosophila and discovered a critical role for importins and exportins, Ran-GTP cycle regulators, nuclear pore components and arginine methylases in mediating DPR toxicity. These findings provide evidence for an important role for nucleocytoplasmic transport in the pathogenic mechanism of c9ALS/FTD. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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