Impact of Airway Inflammation on the Efficacy of CFTR Modulators
Defective CFTR biogenesis and activity in cystic fibrosis airways leads to airway dehydration and impaired mucociliary clearance, resulting in chronic airway infection and inflammation. Most cystic fibrosis patients have at least one copy of the F508del CFTR mutation, which results in a protein reta...
Main Authors: | Carla M. P. Ribeiro, Martina Gentzsch |
---|---|
Format: | Article |
Language: | English |
Published: |
MDPI AG
2021-11-01
|
Series: | Cells |
Subjects: | |
Online Access: | https://www.mdpi.com/2073-4409/10/11/3260 |
Similar Items
-
Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies
by: Martina Gentzsch, et al.
Published: (2021-03-01) -
Evaluation of Fused Pyrrolothiazole Systems as Correctors of Mutant CFTR Protein
by: Virginia Spanò, et al.
Published: (2021-02-01) -
Elexacaftor Mediates the Rescue of F508del CFTR Functional Expression Interacting with MSD2
by: Roberta Bongiorno, et al.
Published: (2023-08-01) -
NBD2 Is Required for the Rescue of Mutant F508del CFTR by a Thiazole-Based Molecule: A Class II Corrector for the Multi-Drug Therapy of Cystic Fibrosis
by: Chiara Brandas, et al.
Published: (2021-09-01) -
Journey on VX-809-Based Hybrid Derivatives towards Drug-like F508del-CFTR Correctors: From Molecular Modeling to Chemical Synthesis and Biological Assays
by: Alice Parodi, et al.
Published: (2022-02-01)