C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport

Repeat expansions in the C9orf72 gene cause amyotrophic lateral sclerosis and frontotemporal dementia characterized by dipeptide-repeat protein (DPR) inclusions. The toxicity associated with two of these DPRs, poly-GR and poly-PR, has been associated with nucleocytoplasmic transport. To investigate...

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Detalles Bibliográficos
Autores principales: Vanneste, Joni, Vercruysse, Thomas, Boeynaems, Steven, Sicart, Adria, Van Damme, Philip, Daelemans, Dirk, Van Den Bosch, Ludo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6823349/
https://www.ncbi.nlm.nih.gov/pubmed/31673013
http://dx.doi.org/10.1038/s41598-019-52035-6
Descripción
Sumario:Repeat expansions in the C9orf72 gene cause amyotrophic lateral sclerosis and frontotemporal dementia characterized by dipeptide-repeat protein (DPR) inclusions. The toxicity associated with two of these DPRs, poly-GR and poly-PR, has been associated with nucleocytoplasmic transport. To investigate the causal role of poly-GR or poly-PR on active nucleocytoplasmic transport, we measured nuclear import and export in poly-GR or poly-PR expressing Hela cells, neuronal-like SH-SY5Y cells and iPSC-derived motor neurons. Our data strongly indicate that poly-GR and poly-PR do not directly impede active nucleocytoplasmic transport.

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