Sigma-1R–Pom121 axis preserves nuclear transport and integrity in poly-PR-induced C9orf72 ALS

Sigma-1R–Pom121 axis preserves nuclear transport and integrity in poly-PR-induced C9orf72 ALS

Nucleocytoplasmic transport disruption contributes to the pathogenesis of C9orf72-associated amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. Among the dipeptide repeat proteins translated from G4C2-repeat RNA, poly-PR is particularly toxic, compromising nuclear envelope integrity an...

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Bibliographic Details
Main Authors: Chun-Yu Lin, Hsuan-Cheng Wu, Ru-Huei Fu, Eddie Feng-Ju Weng, Wen-Chi Hsieh, Tsung-Ping Su, Hsiang-En Wu, Shao-Ming Wang
Format: Article
Language:English
Published: Elsevier 2025-08-01
Series:Neurobiology of Disease
Subjects:
Poly-PR
C9orf72 ALS
Pom121
Sigma-1R
Nucleocytoplasmic transport
Nuclear envelope integrity
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996125002086
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