Original contributionUltrastructure of airway epithelial cell membranes among patients with cystic fibrosis☆
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CFTR rescue with VX-809 and VX-770 favors the repair of primary airway epithelial cell cultures from patients with class II mutations in the presence of Pseudomonas aeruginosa exoproducts
2018, Journal of Cystic FibrosisCitation Excerpt :In cystic fibrosis (CF) airways, chronic inflammation and infections, especially with Pseudomonas aeruginosa (P. aeruginosa) bacteria colonizing most of adult patients, are responsible for progressive lung damage, ultimately leading to respiratory failure. In addition to the commonly observed bronchiectasis, marked alterations of the epithelial tissue have also been reported, including area of severe epithelial shedding, cilia dysmorphologies, tight junction disorganizations as well as tissue remodeling [1–4]. After injury, healthy epithelial tissues sequentially initiate repair mechanisms, in particular cell migration, proliferation and then re-differentiation, in an attempt to restore epithelial integrity and functionality.
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This study was supported in part by National Institutes of Health SCOR Grant HL19171 and PPG Grant HL34322, Environmental Protection Agency Cooperative Agreement CR-807392, and Cystic Fibrosis Foundation RRDP.