Cyclic compression increases F508 Del CFTR expression in ciliated human airway epithelium Journal Article


Authors: Marozkina, N.; Bosch, J.; Cotton, C.; Smith, L.; Seckler, J.; Zaman, K.; Rehman, S.; Periasamy, A.; Gaston, H.; Altawallbeh, G.; Davis, M.; Jones, D. R.; Schilz, R.; Randell, S. H.; Gaston, B.
Article Title: Cyclic compression increases F508 Del CFTR expression in ciliated human airway epithelium
Abstract: The mechanisms by which transepithelial pressure changes observed during exercise and airway clearance can benefit lung health are challenging to study. Here, we have studied 117 mature, fully ciliated airway epithelial cell filters grown at air-liquid interface grown from 10 cystic fibrosis (CF) and 19 control subjects. These were exposed to cyclic increases in apical air pressure of 15 cmH2O for varying times. We measured the effect on proteins relevant to lung health, with a focus on the CF transmembrane regulator (CFTR). Immunofloures-cence and immunoblot data were concordant in demonstrating that air pressure increased F508Del CFTR expression and maturation. This effect was in part dependent on the presence of cilia, on Ca2+ influx, and on formation of nitrogen oxides. These data provide a mechanosensory mechanism by which changes in luminal air pressure, like those observed during exercise and airway clearance, can affect epithelial protein expression and benefit patients with diseases of the airways. © 2019 the American Physiological Society.
Keywords: cystic fibrosis; airway pressure; airway cilia; airway clearance; s-nitrosocysteine
Journal Title: American Journal of Physiology - Lung Cellular and Molecular Physiology
Volume: 317
Issue: 2
ISSN: 1040-0605
Publisher: American Physiological Society  
Date Published: 2019-08-01
Start Page: L247
End Page: L258
Language: English
DOI: 10.1152/ajplung.00020.2019
PUBMED: 31116581
PROVIDER: scopus
PMCID: PMC6734384
DOI/URL:
Notes: Article -- Source: Scopus
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  1. David Randolph Jones
    417 Jones