Title
Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD
Author
Faculty/Department
Faculty of Pharmaceutical, Biomedical and Veterinary Sciences . Biomedical Sciences
Publication type
article
Publication
London :Nature Publishing Group ,
Subject
Engineering sciences. Technology
Source (journal)
Scientific reports. - London, 2011, currens
Volume/pages
6(2016) , 8 p.
ISSN
2045-2322
2045-2322
Article Reference
20877
Carrier
E-only publicatie
Target language
English (eng)
Full text (Publishers DOI)
Affiliation
University of Antwerp
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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Full text (open access)
https://repository.uantwerpen.be/docman/irua/fd408e/132233.pdf
Handle