Targeting nonsense-mediated RNA decay does not increase progranulin levels in the Grn R493X mouse model of frontotemporal dementia.

A common cause of frontotemporal dementia (FTD) are nonsense mutations in the progranulin (GRN) gene. Because nonsense mutations activate the nonsense-mediated RNA decay (NMD) pathway, we sought to inhibit this RNA turnover pathway as a means to increase progranulin levels. Using a knock-in mouse mo...

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Auteurs principaux: Denise M Smith, Michael L Niehoff, Karen Ling, Paymaan Jafar-Nejad, Frank Rigo, Susan A Farr, Miles F Wilkinson, Andrew D Nguyen
Format: Artigo
Langue:Inglês
Publié: Public Library of Science (PLoS) 2023-01-01
Collection:PLoS ONE
Accès en ligne:https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0282822&type=printable
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