ArticleThe European respiratory journal2022
Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties
Article in The European respiratory journal, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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Who cites it
43 citing papers in PubMed, 62 citations in OpenAlex.
- Improved Quality of Life in Children With Cystic Fibrosis Who Received Transmembrane Conductance Regulator Modulators.Acta paediatrica (Oslo, Norway : 1992) · 2026Observational
- The Role of the Respiratory Microbiome in Pneumonia.Clinics in chest medicine · 2026Review
- Therapies for bronchiectasis: mechanisms and potential effects.Frontiers in immunology · 2026Review
- Real-world improvement in ultra-low-dose thoracic computed tomography scores, systemic inflammatory markers and patient-reported outcome measures after elexacaftor/tezacaftor/ivacaftor treatment.ERJ open research · 2025Article
- Ivacaftor ameliorates mucus burden, bacterial load, and inflammation in acute but not chronic P. aeruginosa infection in hG551D rats.Respiratory research · 2024Article
- Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy.European respiratory review : an official journal of the European Respiratory Society · 2024Review
- The lysogenic filamentousPNAS nexus · 2024Article
- Cystic fibrosis.Nature reviews. Disease primers · 2024Review
- Reduced sialylation of airway mucin impairs mucus transport by altering the biophysical properties of mucin.Scientific reports · 2024Article
- Air-liquid intestinal cell culture allowsAPL bioengineering · 2024Article
- Reduced Sialylation of Airway Mucin Impairs Mucus Transport by Altering the Biophysical Properties of Mucin.Research square · 2024Article
- Recent Progress in Nucleic Acid Pulmonary Delivery toward Overcoming Physiological Barriers and Improving Transfection Efficiency.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2024Review
- The role of NaPflugers Archiv : European journal of physiology · 2024Review
- Lytic bacteriophages induce the secretion of antiviral and proinflammatory cytokines from human respiratory epithelial cells.PLoS biology · 2024Article
- Lytic bacteriophages interact with respiratory epithelial cells and induce the secretion of antiviral and proinflammatory cytokines.bioRxiv : the preprint server for biology · 2024Article
- Mucus Structure, Viscoelastic Properties, and Composition in Chronic Respiratory Diseases.International journal of molecular sciences · 2024Review
- Specialized proresolving mediator resolvin E1 corrects the altered cystic fibrosis nasal epithelium cilia beating dynamics.Proceedings of the National Academy of Sciences of the United States of America · 2024Article
- Treatable traits and challenges in the clinical management of non-tuberculous mycobacteria lung disease in people with cystic fibrosis.Respiratory research · 2023Review
- BPIFB1 loss alters airway mucus properties and diminishes mucociliary clearance.American journal of physiology. Lung cellular and molecular physiology · 2023Article
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Corrections and comments
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Authors and funding
17 authors at 2 institutions in 1 country.
Funding
Abstract
questionCystic fibrosis (CF) is characterised by the accumulation of viscous adherent mucus in the lungs. While several hypotheses invoke a direct relationship with cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction ( MATERIALS/
methodsWe characterised a novel cell line (CFTR-KO Calu3 cells) and the responses of human bronchial epithelial (HBE) cells from subjects with G551D or F508del mutations to ivacaftor and elexacaftor-tezacaftor-ivacaftor. A spectrum of assays such as short-circuit currents, quantitative PCR, ASL pH, Western blotting, light scattering/refractometry (size-exclusion chromatography with inline multi-angle light scattering), scanning electron microscopy, percentage solids and particle tracking were performed to determine the impact of CFTR function on mucus properties.
resultsLoss of CFTR function in Calu3 cells resulted in ASL pH acidification and mucus hyperconcentration (dehydration). Modulation of CFTR in CF HBE cells did not affect ASL pH or mucin mRNA expression, but decreased mucus concentration, relaxed mucus network ultrastructure and improved mucus transport. In contrast with modulator-treated cells, a large fraction of airway mucins remained attached to naïve CF cells following short apical washes, as revealed by the use of reducing agents to remove residual mucus from the cell surfaces. Extended hydration, but not buffers alkalised with sodium hydroxide or HCO
conclusionThese results indicate that airway dehydration, not acidic pH and/or low [HCO
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