ArticleNature communications2017
Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens.
Article in Nature communications, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 26 citations in OpenAlex.
- CFTR in alveolar type 1 cells contributes to lung liquid secretion and host defense.JCI insight · 2026Article
- Therapeutic potential of synergistic mucociliary clearance for cystic fibrosis airways by β-adrenergic plus cholinergic agonists.The Journal of clinical investigation · 2026Article
- Reflection confocal microscopy for quantitative assessment of airway surface layer dysregulation and pharmacological rescue in cystic fibrosis under near-physiological conditions.Scientific reports · 2025Article
- Visualization of the hatching of brine shrimp eggs using ultrafast and high-resolution phase-contrast CTs.APL bioengineering · 2025Article
- Hard X-ray imaging and tomography at the Biomedical Imaging and Therapy beamlines of Canadian Light Source.Journal of synchrotron radiation · 2024Article
- MRI overestimates articular cartilage thickness and volume compared to synchrotron radiation phase-contrast imaging.PloS one · 2023Article
- Role of Innate Immune System in Environmental Lung Diseases.Current allergy and asthma reports · 2021Review
- Safety, Tolerability, and Effects of Sodium Bicarbonate Inhalation in Cystic Fibrosis.Clinical drug investigation · 2020Article
- Methods for dynamic synchrotron X-ray respiratory imaging in live animals.Journal of synchrotron radiation · 2020Article
- IL-1β dominates the promucin secretory cytokine profile in cystic fibrosis.The Journal of clinical investigation · 2019Article
- Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis.Science translational medicine · 2019Article
- Nebulized hypertonic saline triggers nervous system-mediated active liquid secretion in cystic fibrosis swine trachea.Scientific reports · 2019Article
- Esomeprazole Increases Airway Surface Liquid pH in Primary Cystic Fibrosis Epithelial Cells.Frontiers in pharmacology · 2018Article
Corrections and comments
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Authors and funding
19 authors at 7 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) channel, which can result in chronic lung disease. The sequence of events leading to lung disease is not fully understood but recent data show that the critical pathogenic event is the loss of the ability to clear bacteria due to abnormal airway surface liquid secretion (ASL). However, whether the inhalation of bacteria triggers ASL secretion and whether this is abnormal in cystic fibrosis has never been tested. Here we show, using a novel synchrotron-based in vivo imaging technique, that wild-type pigs display both a basal and a Toll-like receptor-mediated ASL secretory response to the inhalation of cystic fibrosis relevant bacteria. Both mechanisms fail in CFTR
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