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Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD.
Boeynaems, Steven; Bogaert, Elke; Michiels, Emiel; Gijselinck, Ilse; Sieben, Anne; Jovicic, Ana; De Baets, Greet; Scheveneels, Wendy; Steyaert, Jolien; Cuijt, Ivy; Verstrepen, Kevin J; Callaerts, Patrick; Rousseau, Frederic; Schymkowitz, Joost; Cruts, Marc; Van Broeckhoven, Christine; Van Damme, Philip; Gitler, Aaron D; Robberecht, Wim; Van Den Bosch, Ludo.
Afiliação
  • Boeynaems S; KU Leuven - University of Leuven, Department of Neurosciences, Experimental Neurology and Leuven Research Institute for Neuroscience and Disease (LIND), B-3000 Leuven, Belgium.
  • Bogaert E; VIB, Vesalius Research Center, Laboratory of Neurobiology, B-3000 Leuven, Belgium.
  • Michiels E; KU Leuven - University of Leuven, Department of Neurosciences, Experimental Neurology and Leuven Research Institute for Neuroscience and Disease (LIND), B-3000 Leuven, Belgium.
  • Gijselinck I; VIB, Vesalius Research Center, Laboratory of Neurobiology, B-3000 Leuven, Belgium.
  • Sieben A; KU Leuven - University of Leuven, Department of Neurosciences, Experimental Neurology and Leuven Research Institute for Neuroscience and Disease (LIND), B-3000 Leuven, Belgium.
  • Jovicic A; VIB, Vesalius Research Center, Laboratory of Neurobiology, B-3000 Leuven, Belgium.
  • De Baets G; Department of Molecular Genetics, VIB, B-2610 Antwerp, Belgium.
  • Scheveneels W; Institute Born-Bunge, University of Antwerp, B-2610 Antwerp, Belgium.
  • Steyaert J; Department of Molecular Genetics, VIB, B-2610 Antwerp, Belgium.
  • Cuijt I; Institute Born-Bunge, University of Antwerp, B-2610 Antwerp, Belgium.
  • Verstrepen KJ; Department of Neurology, University Hospital Ghent and University of Ghent, B-9000 Ghent, Belgium.
  • Callaerts P; Department of Genetics, Stanford University School of Medicine, Stanford, CA 94305 USA.
  • Rousseau F; Switch Laboratory, VIB, B-3000 Leuven, Belgium.
  • Schymkowitz J; Switch Laboratory, Department of Cellular and Molecular Medicine, KU Leuven, B-3000 Leuven, Belgium.
  • Cruts M; KU Leuven - University of Leuven, Department of Neurosciences, Experimental Neurology and Leuven Research Institute for Neuroscience and Disease (LIND), B-3000 Leuven, Belgium.
  • Van Broeckhoven C; VIB, Vesalius Research Center, Laboratory of Neurobiology, B-3000 Leuven, Belgium.
  • Van Damme P; KU Leuven - University of Leuven, Department of Neurosciences, Experimental Neurology and Leuven Research Institute for Neuroscience and Disease (LIND), B-3000 Leuven, Belgium.
  • Gitler AD; VIB, Vesalius Research Center, Laboratory of Neurobiology, B-3000 Leuven, Belgium.
  • Robberecht W; Department of Molecular Genetics, VIB, B-2610 Antwerp, Belgium.
  • Van Den Bosch L; Institute Born-Bunge, University of Antwerp, B-2610 Antwerp, Belgium.
Sci Rep ; 6: 20877, 2016 Feb 12.
Article em En | MEDLINE | ID: mdl-26869068
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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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Núcleo Celular / Testes Genéticos / Genes de Insetos / Sequências Repetitivas de Aminoácidos / Dipeptídeos / Drosophila melanogaster / Demência Frontotemporal / Esclerose Lateral Amiotrófica Tipo de estudo: Prognostic_studies Limite: Animals / Humans Idioma: En Ano de publicação: 2016 Tipo de documento: Article
Texto completo
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Núcleo Celular / Testes Genéticos / Genes de Insetos / Sequências Repetitivas de Aminoácidos / Dipeptídeos / Drosophila melanogaster / Demência Frontotemporal / Esclerose Lateral Amiotrófica Tipo de estudo: Prognostic_studies Limite: Animals / Humans Idioma: En Ano de publicação: 2016 Tipo de documento: Article