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

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

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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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Bibliographic Details
Main Authors: Denise M Smith, Michael L Niehoff, Karen Ling, Paymaan Jafar-Nejad, Frank Rigo, Susan A Farr, Miles F Wilkinson, Andrew D Nguyen
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2023-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0282822

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https://doi.org/10.1371/journal.pone.0282822

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