C9orf72-generated poly-GR and poly-PR Do Not Directly Interfere With Nucleocytoplasmic Transport

Joni Vanneste, Thomas Vercruysse, Steven Boeynaems, Adria Sicart, Philip Van Damme, Dirk Daelemans, Ludo Van Den Bosch

Sci Rep. 2019 Oct 31;9(1):15728.

PMID: 31673013

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.

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