Partial Rescue of F508del-CFTR Stability and Trafficking Defects by Double Corrector Treatment

Partial Rescue of F508del-CFTR Stability and Trafficking Defects by Double Corrector Treatment

Deletion of phenylalanine at position 508 (F508del) in the CFTR chloride channel is the most frequent mutation in cystic fibrosis (CF) patients. F508del impairs the stability and folding of the CFTR protein, thus resulting in mistrafficking and premature degradation. F508del-CFTR defects can be over...

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Bibliographic Details
Main Authors: Valeria Capurro, Valeria Tomati, Elvira Sondo, Mario Renda, Anna Borrelli, Cristina Pastorino, Daniela Guidone, Arianna Venturini, Alessandro Giraudo, Sine Mandrup Bertozzi, Ilaria Musante, Fabio Bertozzi, Tiziano Bandiera, Federico Zara, Luis J. V. Galietta, Nicoletta Pedemonte
Format: Article
Language:English
Published: MDPI AG 2021-05-01
Series:International Journal of Molecular Sciences
Subjects:
VX-445
elexacaftor
conformational stability
allosteric folding correction
chloride secretion
primary bronchial cells
Online Access:https://www.mdpi.com/1422-0067/22/10/5262

Internet

https://www.mdpi.com/1422-0067/22/10/5262

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