Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis
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<sup>−</sup> channel. Loss of Cl<sup>−</sup> secretion across the apical membrane of airway lining epi...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2021-03-01
|
| Series: | Genes |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2073-4425/12/3/453 |
| _version_ | 1797540462657011712 |
|---|---|
| author | James A. Reihill Lisa E. J. Douglas S. Lorraine Martin |
| author_facet | James A. Reihill Lisa E. J. Douglas S. Lorraine Martin |
| author_sort | James A. Reihill |
| collection | DOAJ |
| description | 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<sup>−</sup> channel. Loss of Cl<sup>−</sup> 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. |
| first_indexed | 2024-03-10T13:00:32Z |
| format | Article |
| id | doaj.art-f7b468738612435e9fec60f8f1b38faf |
| institution | Directory Open Access Journal |
| issn | 2073-4425 |
| language | English |
| last_indexed | 2024-03-10T13:00:32Z |
| publishDate | 2021-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Genes |
| spelling | doaj.art-f7b468738612435e9fec60f8f1b38faf2023-11-21T11:34:01ZengMDPI AGGenes2073-44252021-03-0112345310.3390/genes12030453Modulation of Ion Transport to Restore Airway Hydration in Cystic FibrosisJames A. Reihill0Lisa E. J. Douglas1S. Lorraine Martin2School of Pharmacy, Queen’s University Belfast, Belfast BT9 7BL, UKSchool of Pharmacy, Queen’s University Belfast, Belfast BT9 7BL, UKSchool of Pharmacy, Queen’s University Belfast, Belfast BT9 7BL, UKCystic 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<sup>−</sup> channel. Loss of Cl<sup>−</sup> 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.https://www.mdpi.com/2073-4425/12/3/453cystic fibrosision channelcystic fibrosis transmembrane conductance regulator (CFTR)epithelial sodium channel (ENaC)transmembrane member 16A (TMEM16A)big potassium (BK) |
| spellingShingle | James A. Reihill Lisa E. J. Douglas S. Lorraine Martin Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis Genes cystic fibrosis ion channel cystic fibrosis transmembrane conductance regulator (CFTR) epithelial sodium channel (ENaC) transmembrane member 16A (TMEM16A) big potassium (BK) |
| title | Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis |
| title_full | Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis |
| title_fullStr | Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis |
| title_full_unstemmed | Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis |
| title_short | Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis |
| title_sort | modulation of ion transport to restore airway hydration in cystic fibrosis |
| topic | cystic fibrosis ion channel cystic fibrosis transmembrane conductance regulator (CFTR) epithelial sodium channel (ENaC) transmembrane member 16A (TMEM16A) big potassium (BK) |
| url | https://www.mdpi.com/2073-4425/12/3/453 |
| work_keys_str_mv | AT jamesareihill modulationofiontransporttorestoreairwayhydrationincysticfibrosis AT lisaejdouglas modulationofiontransporttorestoreairwayhydrationincysticfibrosis AT slorrainemartin modulationofiontransporttorestoreairwayhydrationincysticfibrosis |