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Volume 12
Issue 3
10.3390/genes12030453
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Review

Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis

by
James A. Reihill
*,
Lisa E. J. Douglas
and
S. Lorraine Martin
School of Pharmacy, Queen’s University Belfast, Belfast BT9 7BL, UK
*
Author to whom correspondence should be addressed.
Genes 2021, 12(3), 453; https://doi.org/10.3390/genes12030453
Submission received: 25 February 2021 / Revised: 11 March 2021 / Accepted: 19 March 2021 / Published: 22 March 2021
(This article belongs to the Special Issue Phenotypic Variability of Cystic Fibrosis: New Challenges)
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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.
Keywords: cystic fibrosis; ion channel; cystic fibrosis transmembrane conductance regulator (CFTR); epithelial sodium channel (ENaC); transmembrane member 16A (TMEM16A); big potassium (BK); airway hydration cystic fibrosis; ion channel; cystic fibrosis transmembrane conductance regulator (CFTR); epithelial sodium channel (ENaC); transmembrane member 16A (TMEM16A); big potassium (BK); airway hydration

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MDPI and ACS Style

Reihill, J.A.; Douglas, L.E.J.; Martin, S.L. Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis. Genes 2021, 12, 453. https://doi.org/10.3390/genes12030453

AMA Style

Reihill JA, Douglas LEJ, Martin SL. Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis. Genes. 2021; 12(3):453. https://doi.org/10.3390/genes12030453

Chicago/Turabian Style

Reihill, James A., Lisa E. J. Douglas, and S. Lorraine Martin. 2021. "Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis" Genes 12, no. 3: 453. https://doi.org/10.3390/genes12030453

APA Style

Reihill, J. A., Douglas, L. E. J., & Martin, S. L. (2021). Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis. Genes, 12(3), 453. https://doi.org/10.3390/genes12030453

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