UPF1 reduces C9orf72 HRE-induced neurotoxicity in the absence of nonsense-mediated decay dysfunction

UPF1 reduces C9orf72 HRE-induced neurotoxicity in the absence of nonsense-mediated decay dysfunction

Summary: Multiple cellular pathways have been suggested to be altered by the C9orf72 GGGGCC (G4C2) hexanucleotide repeat expansion (HRE), including aspects of RNA regulation such as nonsense-mediated decay (NMD). Here, we investigate the role that overexpression of UPF1, a protein involved in NMD, p...

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Bibliographic Details
Main Authors: Benjamin L. Zaepfel, Zhe Zhang, Kirstin Maulding, Alyssa N. Coyne, Weiwei Cheng, Lindsey R. Hayes, Thomas E. Lloyd, Shuying Sun, Jeffrey D. Rothstein
Format: Article
Language:English
Published: Elsevier 2021-03-01
Series:Cell Reports
Subjects:
amyotrophic lateral sclerosis
C9ORF72
nonsense-mediated decay
UPF1
frontotemporal dementia
neurotoxicity
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124721002394

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http://www.sciencedirect.com/science/article/pii/S2211124721002394

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