Targeting nonsense-mediated RNA decay does not increase progranulin levels in the GrnR493X 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...

Full description

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://www.ncbi.nlm.nih.gov/pmc/articles/PMC9997918/?tool=EBI