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

doi:10.1016/j.celrep.2021.108925

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

School of Medicine

Research output: Contribution to journal › Article › peer-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 language	English (US)
Article number	108925
Journal	Cell Reports
Volume	34
Issue number	13
DOIs	https://doi.org/10.1016/j.celrep.2021.108925
State	Published - Mar 30 2021

Keywords

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

ASJC Scopus subject areas

General Biochemistry, Genetics and Molecular Biology

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10.1016/j.celrep.2021.108925

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title = "UPF1 reduces C9orf72 HRE-induced neurotoxicity in the absence of nonsense-mediated decay dysfunction",

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.",

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AU - Zaepfel, Benjamin L.

AU - Zhang, Zhe

AU - Maulding, Kirstin

AU - Coyne, Alyssa N.

AU - Cheng, Weiwei

AU - Hayes, Lindsey R.

AU - Lloyd, Thomas E.

AU - Sun, Shuying

AU - Rothstein, Jeffrey D.

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AB - 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.

KW - C9ORF72

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KW - amyotrophic lateral sclerosis

KW - frontotemporal dementia

KW - induced pluripotent cells

KW - neurons

KW - neurotoxicity

KW - nonsense-mediated decay

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

Abstract

Keywords

ASJC Scopus subject areas

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