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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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
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author Vanneste, Joni
Vercruysse, Thomas
Boeynaems, Steven
Sicart, Adria
Van Damme, Philip
Daelemans, Dirk
Van Den Bosch, Ludo
author_facet Vanneste, Joni
Vercruysse, Thomas
Boeynaems, Steven
Sicart, Adria
Van Damme, Philip
Daelemans, Dirk
Van Den Bosch, Ludo
author_sort Vanneste, Joni
collection PubMed
description 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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spelling pubmed-68233492019-11-12 C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport Vanneste, Joni Vercruysse, Thomas Boeynaems, Steven Sicart, Adria Van Damme, Philip Daelemans, Dirk Van Den Bosch, Ludo Sci Rep Article 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. Nature Publishing Group UK 2019-10-31 /pmc/articles/PMC6823349/ /pubmed/31673013 http://dx.doi.org/10.1038/s41598-019-52035-6 Text en © The Author(s) 2019 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
spellingShingle Article
Vanneste, Joni
Vercruysse, Thomas
Boeynaems, Steven
Sicart, Adria
Van Damme, Philip
Daelemans, Dirk
Van Den Bosch, Ludo
C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport
title C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport
title_full C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport
title_fullStr C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport
title_full_unstemmed C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport
title_short C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport
title_sort c9orf72-generated poly-gr and poly-pr do not directly interfere with nucleocytoplasmic transport
topic Article
url 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
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