Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis

James A. Reihill*, Lisa E.J. Douglas, S. Lorraine Martin

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

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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.
Original languageEnglish
Article number453
JournalGenes
Volume12
Issue number3
DOIs
Publication statusPublished - 22 Mar 2021

Keywords

  • cystic fibrosis
  • ion channels
  • ENaC
  • CFTR
  • hydration
  • mucociliary clearance

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