ArticleJCI insight2017
Defective postsecretory maturation of MUC5B mucin in cystic fibrosis airways.
Article in JCI insight, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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.
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Who cites it
25 citing papers in PubMed, 40 citations in OpenAlex.
- Increased low-molecular-weight mucins in muco-obstructive airway disease limitInfection and immunity · 2026Article
- Host- and microbial-mediated mucin degradation differentially shape Pseudomonas aeruginosa physiology and gene expression.PLoS pathogens · 2025Article
- Normalization of Muc5b ameliorates airway mucus plugging during persistentAmerican journal of physiology. Lung cellular and molecular physiology · 2024Article
- The Tmem16a chloride channel is required for mucin maturation after secretion from goblet-like cells in the Xenopus tropicalis tadpole skin.Scientific reports · 2024Article
- Acquired dysfunction of CFTR underlies cystic fibrosis-like disease of the canine gallbladder.American journal of physiology. Gastrointestinal and liver physiology · 2024Article
- Reduced sialylation of airway mucin impairs mucus transport by altering the biophysical properties of mucin.Scientific reports · 2024Article
- Reduced Sialylation of Airway Mucin Impairs Mucus Transport by Altering the Biophysical Properties of Mucin.Research square · 2024Article
- 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
- CFTR dysfunction leads to defective bacterial eradication on cystic fibrosis airways.Frontiers in physiology · 2024Review
- SNSP113 (PAAG) improves mucociliary transport and lung pathology in the Scnn1b-Tg murine model of CF lung disease.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2023Article
- Muc5b Contributes to Mucus Abnormality in Rat Models of Cystic Fibrosis.Frontiers in physiology · 2022Article
- CFTR Protein: Not Just a Chloride Channel?Cells · 2021Review
- Mucolytic bacteria: prevalence in various pathological diseases.World journal of microbiology & biotechnology · 2021Review
- Airway Surface Liquid pH Regulation in Airway Epithelium Current Understandings and Gaps in Knowledge.International journal of molecular sciences · 2021Review
- Intravital microscopic optical coherence tomography imaging to assess mucus-mobilizing interventions for muco-obstructive lung disease in mice.American journal of physiology. Lung cellular and molecular physiology · 2020Article
- Surfing motility is a complex adaptation dependent on the stringent stress response in Pseudomonas aeruginosa LESB58.PLoS pathogens · 2020Article
- The MUC5B mucin polymer is dominated by repeating structural motifs and its topology is regulated by calcium and pH.Scientific reports · 2019Article
- The C-terminal dimerization domain of the respiratory mucin MUC5B functions in mucin stability and intracellular packaging before secretion.The Journal of biological chemistry · 2019Article
- Mucus, mucins, and cystic fibrosis.Pediatric pulmonology · 2019Review
- Small molecule-facilitated anion transporters in cells for a novel therapeutic approach to cystic fibrosis.British journal of pharmacology · 2019Article
Corrections and comments
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Authors and funding
11 authors at 2 institutions in 1 country.
Funding
Abstract
In cystic fibrosis (CF), airway mucus becomes thick and viscous, and its clearance from the airways is impaired. The gel-forming mucins undergo an ordered "unpacking/maturation" process after granular release that requires an optimum postsecretory environment, including hydration and pH. We hypothesized that this unpacking process is compromised in the CF lung due to abnormal transepithelial fluid transport that reduces airway surface hydration and alters ionic composition. Using human tracheobronchial epithelial cells derived from non-CF and CF donors and mucus samples from human subjects and domestic pigs, we investigated the process of postsecretory mucin unfolding/maturation, how these processes are defective in CF airways, and the probable mechanism underlying defective unfolding. First, we found that mucins released into a normal lung environment transform from a compact granular form to a linear form. Second, we demonstrated that this maturation process is defective in the CF airway environment. Finally, we demonstrated that independent of HCO
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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.