Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD
- Author
- S Boeynaems, Elke Bogaert (UGent) , E Michiels, I Gijselinck, Anne Sieben (UGent) , A Jovičić, G De Baets, W Scheveneels, J Steyaert, I Cuijt, KJ Verstrepen, P Callaerts, F Rousseau, J Schymkowitz, M Cruts, C Van Broeckhoven, P Van Damme, AD Gitler, W Robberecht and L Van Den Bosch
- Organization
- Abstract
- 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.
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Citation
Please use this url to cite or link to this publication: http://hdl.handle.net/1854/LU-01GXBG5QV05KK365NBHH7TN1PM
- MLA
- Boeynaems, S., et al. “Drosophila Screen Connects Nuclear Transport Genes to DPR Pathology in C9ALS/FTD.” SCIENTIFIC REPORTS, vol. 6, 2016, doi:10.1038/srep20877.
- APA
- Boeynaems, S., Bogaert, E., Michiels, E., Gijselinck, I., Sieben, A., Jovičić, A., … Van Den Bosch, L. (2016). Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD. SCIENTIFIC REPORTS, 6. https://doi.org/10.1038/srep20877
- Chicago author-date
- Boeynaems, S, Elke Bogaert, E Michiels, I Gijselinck, Anne Sieben, A Jovičić, G De Baets, et al. 2016. “Drosophila Screen Connects Nuclear Transport Genes to DPR Pathology in C9ALS/FTD.” SCIENTIFIC REPORTS 6. https://doi.org/10.1038/srep20877.
- Chicago author-date (all authors)
- Boeynaems, S, Elke Bogaert, E Michiels, I Gijselinck, Anne Sieben, A Jovičić, G De Baets, W Scheveneels, J Steyaert, I Cuijt, KJ Verstrepen, P Callaerts, F Rousseau, J Schymkowitz, M Cruts, C Van Broeckhoven, P Van Damme, AD Gitler, W Robberecht, and L Van Den Bosch. 2016. “Drosophila Screen Connects Nuclear Transport Genes to DPR Pathology in C9ALS/FTD.” SCIENTIFIC REPORTS 6. doi:10.1038/srep20877.
- Vancouver
- 1.Boeynaems S, Bogaert E, Michiels E, Gijselinck I, Sieben A, Jovičić A, et al. Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD. SCIENTIFIC REPORTS. 2016;6.
- IEEE
- [1]S. Boeynaems et al., “Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD,” SCIENTIFIC REPORTS, vol. 6, 2016.
@article{01GXBG5QV05KK365NBHH7TN1PM,
abstract = {{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.}},
articleno = {{20877}},
author = {{Boeynaems, S and Bogaert, Elke and Michiels, E and Gijselinck, I and Sieben, Anne and Jovičić, A and De Baets, G and Scheveneels, W and Steyaert, J and Cuijt, I and Verstrepen, KJ and Callaerts, P and Rousseau, F and Schymkowitz, J and Cruts, M and Van Broeckhoven, C and Van Damme, P and Gitler, AD and Robberecht, W and Van Den Bosch, L}},
issn = {{2045-2322}},
journal = {{SCIENTIFIC REPORTS}},
language = {{eng}},
pages = {{8}},
title = {{Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD}},
url = {{http://doi.org/10.1038/srep20877}},
volume = {{6}},
year = {{2016}},
}
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