Cystic Fibrosis Transmembrane Conductance Regulator Folding Mutations Reveal Differences in Corrector Efficacy Linked to Increases in Immature Cystic Fibrosis Transmembrane Conductance Regulator Expression

Cystic Fibrosis Transmembrane Conductance Regulator Folding Mutations Reveal Differences in Corrector Efficacy Linked to Increases in Immature Cystic Fibrosis Transmembrane Conductance Regulator Expression

Background: Most cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that lead to protein misfolding and degradation by the ubiquitin–proteasome system. Previous studies demonstrated that PIAS4 facilitates the modification of wild-type (WT) a...

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Bibliographic Details
Main Authors: Kathryn W. Peters, Xiaoyan Gong, Raymond A. Frizzell
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-10-01
Series:Frontiers in Physiology
Subjects:
cystic fibrosis
CFTR
mutations
corrector efficacy
SUMOylation
Online Access:https://www.frontiersin.org/articles/10.3389/fphys.2021.695767/full

Internet

https://www.frontiersin.org/articles/10.3389/fphys.2021.695767/full

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