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Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models

In a subgroup of patients with amyotrophic lateral sclerosis (ALS)/Frontotemporal dementia (FTD), the (G4C2)-RNA repeat expansion from C9orf72 chromosome binds to the Ran-activating protein (RanGAP) at the nuclear pore, resulting in nucleocytoplasmic transport deficit and accumulation of Ran in the...

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Detalles Bibliográficos
Autores principales: Lee, Pin-Tse, Liévens, Jean-Charles, Wang, Shao-Ming, Chuang, Jian-Ying, Khalil, Bilal, Wu, Hsiang-en, Chang, Wen-Chang, Maurice, Tangui, Su, Tsung-Ping
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7642387/
https://www.ncbi.nlm.nih.gov/pubmed/33149115
http://dx.doi.org/10.1038/s41467-020-19396-3