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Article

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

Cell reports - 30 Mar 2021

Zaepfel Benjamin L, Zhang Zhe, Maulding Kirstin, Coyne Alyssa N, Cheng Weiwei, Hayes Lindsey R, Lloyd Thomas E, Sun Shuying, Rothstein Jeffrey D

Abstract excerpt

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, plays in mitigating neurotoxicity in multiple models of C9orf72 ALS/FTD. First, we show that NMD is not altered in our...

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Identifiers

PMID: 33789100
DOI: 10.1016/j.celrep.2021.108925
PMCID: PMC8063722

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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