Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis
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Updated: Nov 10, 2025

Real-Time, Semi-Automated Fluorescent Measurement of the Airway Surface Liquid pH of Primary Human Airway Epithelial Cells
Published on: June 13, 2019
James A Reihill1, Lisa E J Douglas1, S Lorraine Martin1
1School of Pharmacy, Queen's University Belfast, Belfast BT9 7BL, UK.
Abstract:
Cystic fibrosis (CF) is a life-limiting genetic disorder caused by loss-of-function mutations in the gene which codes for the CF transmembrane conductance regulator (CFTR) Cl- channel. Loss of Cl- secretion across the apical membrane of airway lining epithelial cells results in dehydration of the airway surface liquid (ASL) layer which impairs mucociliary clearance (MCC), and as a consequence promotes bacterial infection and inflammation of the airways. Interventions that restore airway hydration are known to improve MCC. Here we review the ion channels present at the luminal surface of airway epithelial cells that may be targeted to improve airway hydration and MCC in CF airways.
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