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

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

Research output: Contribution to journalArticlepeer-review

Abstract

Zaepfel et al. show that UPF1 is neuroprotective in the context of C9-ALS. This neuroprotection is observed in multiple in vitro and in vivo models of C9-ALS. UPF1 mitigates toxicity independently of its role in nonsense-mediated decay but is dependent on its known RNA-binding and helicase activity.

Original languageEnglish (US)
Article number108925
JournalCell Reports
Volume34
Issue number13
DOIs
StatePublished - Mar 30 2021

Keywords

  • C9ORF72
  • UPF1
  • amyotrophic lateral sclerosis
  • frontotemporal dementia
  • induced pluripotent cells
  • neurons
  • neurotoxicity
  • nonsense-mediated decay

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

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