ArticleAmerican journal of physiology. Lung cellular and molecular physiology2016
Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense.
Article in American journal of physiology. Lung cellular and molecular physiology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers.
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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.
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Who cites it
38 citing papers in PubMed, 52 citations in OpenAlex.
- TSLP impairs mucociliary transport by inhibiting JAK signaling in both central and peripheral airways.Pflugers Archiv : European journal of physiology · 2026Article
- Antiviral defenses are diminished at birth in cystic fibrosis pig airways.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2026Article
- Microenvironmental effects of a non-antibiotic therapy for a chronic Polymicrobial infection Alter microbial physiology, competition, and virulence.The ISME journal · 2026Article
- CFTR as a therapeutic target for severe lung infection.American journal of physiology. Lung cellular and molecular physiology · 2025Review
- Toward in vivo bronchoscopic functional CFTR assessment using a short circuit current measurement probe.American journal of physiology. Lung cellular and molecular physiology · 2025Article
- Unveiling regulatory variants in the blood transcriptome and their association with immunity traits in pigs.Frontiers in immunology · 2025Article
- Mucociliary clearance is impaired in small airways of cystic fibrosis pigs.American journal of physiology. Lung cellular and molecular physiology · 2024Article
- CFTR dysfunction leads to defective bacterial eradication on cystic fibrosis airways.Frontiers in physiology · 2024Review
- Mechanisms by which the cystic fibrosis transmembrane conductance regulator may influence SARS-CoV-2 infection and COVID-19 disease severity.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2023Article
- Targeting ATP12A, a Nongastric Proton Pump α Subunit, for Idiopathic Pulmonary Fibrosis Treatment.American journal of respiratory cell and molecular biology · 2023Article
- Biotin-dependent cell envelope remodelling is required for Mycobacterium abscessus survival in lung infection.Nature microbiology · 2023Article
- Identification of transcriptional regulatory variants in pig duodenum, liver, and muscle tissues.GigaScience · 2022Article
- Mucins and CFTR: Their Close Relationship.International journal of molecular sciences · 2022Review
- Bicarbonate Effects on Antibacterial Immunity and Mucus Glycobiology in the Cystic Fibrosis Lung: A Review With Selected Experimental Observations.Infectious microbes & diseases · 2022Article
- A Single-Cell Atlas of Large and Small Airways at Birth in a Porcine Model of Cystic Fibrosis.American journal of respiratory cell and molecular biology · 2022Article
- Advances in pig models of human diseases.Animal models and experimental medicine · 2022Review
- Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs.Proceedings of the National Academy of Sciences of the United States of America · 2022Article
- Cystic Fibrosis and the Cells of the Airway Epithelium: What Are Ionocytes and What Do They Do?Annual review of pathology · 2022Review
- Application of the transgenic pig model in biomedical research: A review.Frontiers in cell and developmental biology · 2022Review
- Published anti-SARS-CoV-2 in vitro hits share common mechanisms of action that synergize with antivirals.Briefings in bioinformatics · 2021Article
Corrections and comments
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Authors and funding
14 authors at 1 institution in 1 country.
Funding
Abstract
While pathological and clinical data suggest that small airways are involved in early cystic fibrosis (CF) lung disease development, little is known about how the lack of cystic fibrosis transmembrane conductance regulator (CFTR) function contributes to disease pathogenesis in these small airways. Large and small airway epithelia are exposed to different airflow velocities, temperatures, humidity, and CO2 concentrations. The cellular composition of these two regions is different, and small airways lack submucosal glands. To better understand the ion transport properties and impacts of lack of CFTR function on host defense function in small airways, we adapted a novel protocol to isolate small airway epithelial cells from CF and non-CF pigs and established an organotypic culture model. Compared with non-CF large airways, non-CF small airway epithelia cultures had higher Cl(-) and bicarbonate (HCO3 (-)) short-circuit currents and higher airway surface liquid (ASL) pH under 5% CO2 conditions. CF small airway epithelia were characterized by minimal Cl(-) and HCO3 (-) transport and decreased ASL pH, and had impaired bacterial killing compared with non-CF small airways. In addition, CF small airway epithelia had a higher ASL viscosity than non-CF small airways. Thus, the activity of CFTR is higher in the small airways, where it plays a role in alkalinization of ASL, enhancement of antimicrobial activity, and lowering of mucus viscosity. These data provide insight to explain why the small airways are a susceptible site for the bacterial colonization.
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