ArticleAmerican journal of physiology. Lung cellular and molecular physiology2012
CFTR is required for maximal transepithelial liquid transport in pig alveolar epithelia.
Article in American journal of physiology. Lung cellular and molecular physiology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 34 citations in OpenAlex.
- Quantitative imaging of airway liquid absorption in cystic fibrosis.The European respiratory journal · 2014Trial
- Trial
- CFTR in alveolar type 1 cells contributes to lung liquid secretion and host defense.JCI insight · 2026Article
- Fuelling repair: metabolic regulation of alveolar type 2 cell fate in lung regeneration and disease.Disease models & mechanisms · 2026Review
- CFTR as a therapeutic target for severe lung infection.American journal of physiology. Lung cellular and molecular physiology · 2025Review
- Rescue of alveolar wall liquid secretion blocks fatal lung injury due to influenza-staphylococcal coinfection.The Journal of clinical investigation · 2023Article
- Article
- New Insights into the Alveolar Epithelium as a Driver of Acute Respiratory Distress Syndrome.Biomolecules · 2022Review
- V-Type ATPase Mediates Airway Surface Liquid Acidification in Pig Small Airway Epithelial Cells.American journal of respiratory cell and molecular biology · 2021Article
- Pathways in the Pathophysiology of Coronavirus 19 Lung Disease Accessible to Prevention and Treatment.Frontiers in physiology · 2020Article
- Article
- Characterization of air-liquid interface culture of A549 alveolar epithelial cells.Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologica · 2017Article
- Cystic Fibrosis Transmembrane Conductance Regulator Potentiation as a Therapeutic Strategy for Pulmonary Edema: A Proof-of-Concept Study in Pigs.Critical care medicine · 2017Article
- Differential expression of intestinal ion transporters and water channel aquaporins in young piglets challenged with enterotoxigenicJournal of animal science · 2017Article
- Article
- Loss of carbonic anhydrase XII function in individuals with elevated sweat chloride concentration and pulmonary airway disease.Human molecular genetics · 2016Article
- Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense.American journal of physiology. Lung cellular and molecular physiology · 2016Article
- Simulation of Cl(-) Secretion in Epithelial Tissues: New Methodology Estimating Activity of Electro-Neutral Cl(-) Transporter.Frontiers in physiology · 2015Article
- CFTR and lung homeostasis.American journal of physiology. Lung cellular and molecular physiology · 2014Review
- A critical review of the American Journal of Physiology-Lung Cellular and Molecular Physiology: 2012-2015.American journal of physiology. Lung cellular and molecular physiology · 2014Review
Corrections and comments
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Authors and funding
14 authors at 2 institutions in 1 country.
Funding
Abstract
A balance between alveolar liquid absorption and secretion is critical for maintaining optimal alveolar subphase liquid height and facilitating gas exchange in the alveolar space. However, the role of cystic fibrosis transmembrane regulator protein (CFTR) in this homeostatic process has remained elusive. Using a newly developed porcine model of cystic fibrosis, in which CFTR is absent, we investigated ion transport properties and alveolar liquid transport in isolated type II alveolar epithelial cells (T2AECs) cultured at the air-liquid interface. CFTR was distributed exclusively to the apical surface of cultured T2AECs. Alveolar epithelia from CFTR(-/-) pigs failed to increase liquid absorption in response to agents that increase cAMP, whereas cAMP-stimulated liquid absorption in CFTR(+/-) epithelia was similar to that in CFTR(+/+) epithelia. Expression of recombinant CFTR restored stimulated liquid absorption in CFTR(-/-) T2AECs but had no effect on CFTR(+/+) epithelia. In ex vivo studies of nonperfused lungs, stimulated liquid absorption was defective in CFTR(-/-) alveolar epithelia but similar between CFTR(+/+) and CFTR(+/-) epithelia. When epithelia were studied at the air-liquid interface, elevating cAMP levels increased subphase liquid height in CFTR(+/+) but not in CFTR(-/-) T2AECs. Our findings demonstrate that CFTR is required for maximal liquid absorption under cAMP stimulation, but it is not the rate-limiting factor. Furthermore, our data define a role for CFTR in liquid secretion by T2AECs. These insights may help to develop new treatment strategies for pulmonary edema and respiratory distress syndrome, diseases in which lung liquid transport is disrupted.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.