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

ABSTRACT: 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.

SUBMITTER: Vanneste J

PROVIDER: S-EPMC6823349 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature

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

Vanneste Joni J Vercruysse Thomas T Boeynaems Steven S Boeynaems Steven S Sicart Adria A Van Damme Philip P Daelemans Dirk D Van Den Bosch Ludo L

Scientific reports 20191031 1

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-der ...[more]

PMID: 31673013

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