ArticleThe Journal of clinical investigation2014
Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure.
Article in The Journal of clinical investigation, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05392855 (Symptom Based Performance of Airway Clearance Therapy After Starting Highly Effective CFTR Modulator Therapy for Cystic Fibrosis), which is not on this map. Cited by 207 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Symptom Based Performance of Airway Clearance Therapy After Starting Highly Effective CFTR Modulator Therapy for Cystic Fibrosis
Who cites it
207 citing papers in PubMed, 1 synthesis or guideline pooled it, 338 citations in OpenAlex.
- Impact of N-Acetylcysteine on Mucus Hypersecretion in the Airways: A Systematic Review.International journal of chronic obstructive pulmonary disease · 2024Pooled it
- Intranasal micro-optical coherence tomography imaging for cystic fibrosis studies.Science translational medicine · 2019Trial
- Hypertonic saline has a prolonged effect on mucociliary clearance in adults with cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2018Trial
- Gamma tocopherol-enriched supplement reduces sputum eosinophilia and endotoxin-induced sputum neutrophilia in volunteers with asthma.The Journal of allergy and clinical immunology · 2018Trial
- Host-pathogen pyrimidine metabolism drives cystic fibrosis lung disease.Nature communications · 2026Article
- Article
- Biomechanical and Aerodynamic Modulation for Sinonasal Homeostasis in Craniofacial Orthopedics: A Comprehensive Review of RAMPA Therapy.Biomimetics (Basel, Switzerland) · 2026Review
- A novel stress response pathway mediates biofilm architecture in Pseudomonas aeruginosa.PLoS pathogens · 2026Article
- Cilia-independent gas-liquid transport, a third mechanism mediating airway mucus clearance.The Journal of clinical investigation · 2026Article
- Contribution of two-component regulatory systems to the acute-to-chronic infection transition ofJournal of bacteriology · 2026Review
- Anaerobic microbiota promote pathogen association with the airway epithelium.Journal of medical microbiology · 2026Article
- Bronchial epithelial-derived exosomal SLC6A14 promotes airway inflammation and mucus hypersecretion via the ZFP36L1/IL-8 axis.Functional & integrative genomics · 2026Article
- A Quantitative Lung Mucin Score to Identify Chronic Bronchitis.NEJM evidence · 2026Article
- Elasticity promotes directional transport ofSoft matter · 2026Article
- Airway mucus in infection.Frontiers in physiology · 2026Review
- Extracellular vesicle-based delivery to airway basal cells for durable gene therapy in cystic fibrosis.Frontiers in bioengineering and biotechnology · 2026Review
- SputOMICs identifies common and distinct markers in cystic fibrosis and chronic obstructive pulmonary disease.Scientific reports · 2025Article
- Single cell and spatial characterization of the human pancreas reveals drivers of beta cell dysfunction in cystic fibrosis.bioRxiv : the preprint server for biology · 2025Article
- Farnesol emulsion for elimination ofBiofilm · 2025Article
- Spatial Pharmacokinetic and Pharmacodynamic Modeling in Airway Mucus.Clinical pharmacokinetics · 2025Article
147 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
13 authors at 3 institutions in 1 country.
Funding
Abstract
The pathogenesis of mucoinfective lung disease in cystic fibrosis (CF) patients likely involves poor mucus clearance. A recent model of mucus clearance predicts that mucus flow depends on the relative mucin concentration of the mucus layer compared with that of the periciliary layer; however, mucin concentrations have been difficult to measure in CF secretions. Here, we have shown that the concentration of mucin in CF sputum is low when measured by immunologically based techniques, and mass spectrometric analyses of CF mucins revealed mucin cleavage at antibody recognition sites. Using physical size exclusion chromatography/differential refractometry (SEC/dRI) techniques, we determined that mucin concentrations in CF secretions were higher than those in normal secretions. Measurements of partial osmotic pressures revealed that the partial osmotic pressure of CF sputum and the retained mucus in excised CF lungs were substantially greater than the partial osmotic pressure of normal secretions. Our data reveal that mucin concentration cannot be accurately measured immunologically in proteolytically active CF secretions; mucins are hyperconcentrated in CF secretions; and CF secretion osmotic pressures predict mucus layer-dependent osmotic compression of the periciliary liquid layer in CF lungs. Consequently, mucin hypersecretion likely produces mucus stasis, which contributes to key infectious and inflammatory components of CF lung disease.
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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.