The F508del-CFTR trafficking correctors elexacaftor and tezacaftor are CFTR-independent Ca2+-mobilizing agonists normalizing abnormal Ca2+ levels in human airway epithelial cells

The F508del-CFTR trafficking correctors elexacaftor and tezacaftor are CFTR-independent Ca2+-mobilizing agonists normalizing abnormal Ca2+ levels in human airway epithelial cells

Abstract Background Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) channel. For people with CF (pwCF) affected by the most common pathogenic variant F508del, a tritherapy, named Trikafta/Kaftrio (ETI: elexacaftor (VX-44...

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Bibliographic Details
Main Authors: Manuella Lévêque, Sandra Mirval, Christine Barrault, Isabelle Fixe, Christelle Coraux, Edouard Sage, Frédéric Becq, Clarisse Vandebrouck
Format: Article
Language:English
Published: BMC 2024-12-01
Series:Respiratory Research
Subjects:
Cystic fibrosis
ETI
Calcium
SERCA
CFTR
Endoplasmic reticulum
Online Access:https://doi.org/10.1186/s12931-024-03059-8

Internet

https://doi.org/10.1186/s12931-024-03059-8

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