A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay

A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay

Nonsense mutations that lead to the insertion of a premature termination codon (PTC) in the cystic fibrosis transmembrane conductance regulator (CFTR) transcript affect 11% of patients with cystic fibrosis (CF) worldwide and are associated with severe disease phenotype. While CF rat models have cont...

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Bibliographic Details
Main Authors: Jyoti Sharma, Joseph Abbott, Lauren Klaskala, Guojun Zhao, Susan E. Birket, Steven M. Rowe
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-12-01
Series:Frontiers in Physiology
Subjects:
cystic fibrosis
nonsense mutation G542X
rat model
rat epithelial cells
translational readthrough
Online Access:https://www.frontiersin.org/articles/10.3389/fphys.2020.611294/full

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