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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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Detalles Bibliográficos
Autores principales: Smith, Denise M., Niehoff, Michael L., Ling, Karen, Jafar-Nejad, Paymaan, Rigo, Frank, Farr, Susan A., Wilkinson, Miles F., Nguyen, Andrew D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9997918/
https://www.ncbi.nlm.nih.gov/pubmed/36893203
http://dx.doi.org/10.1371/journal.pone.0282822