cAMP-activated Ca2+ signaling is required for CFTR-mediated serous cell fluid secretion in porcine and human airways

RJ Lee, JK Foskett - The Journal of clinical investigation, 2010 - Am Soc Clin Investig
Cystic fibrosis (CF), which is caused by mutations in CFTR, affects many tissues, including
the lung. Submucosal gland serous acinar cells are primary sites of fluid secretion and …

Quantitative Proteomics Reveals an Altered Cystic Fibrosis In Vitro Bronchial Epithelial Secretome

JR Peters-Hall, KJ Brown, DK Pillai… - American journal of …, 2015 - atsjournals.org
Alterations in epithelial secretions and mucociliary clearance contribute to chronic bacterial
infection in cystic fibrosis (CF) lung disease, but whether CF lungs are unchanged in the …

Pseudomonas aeruginosa inhibits endocytic recycling of CFTR in polarized human airway epithelial cells

A Swiatecka-Urban… - … of physiology-cell …, 2006 - journals.physiology.org
The most common mutation in the CFTR gene in individuals with cystic fibrosis (CF), ΔF508,
leads to the absence of CFTR Cl− channels in the apical plasma membrane, which in turn …

Physiology of epithelial chloride and fluid secretion

RA Frizzell, JW Hanrahan - Cold Spring …, 2012 - perspectivesinmedicine.cshlp.org
Epithelial salt and water secretion serves a variety of functions in different organ systems,
such as the airways, intestines, pancreas, and salivary glands. In cystic fibrosis (CF), the …

Mucus secretion by single tracheal submucosal glands from normal and cystic fibrosis transmembrane conductance regulator knockout mice

JP Ianowski, JY Choi, JJ Wine… - The Journal of …, 2007 - Wiley Online Library
Submucosal glands line the cartilaginous airways and produce most of the antimicrobial
mucus that keeps the airways sterile. The glands are defective in cystic fibrosis (CF), but how …

Airway epithelial cell inflammatory signalling in cystic fibrosis

J Jacquot, O Tabary, P Le Rouzic, A Clement - The international journal of …, 2008 - Elsevier
Cystic fibrosis (CF) is the most common lethal monogenic disorder in Caucasians, estimated
to affect one out of 2500–4000 new-borns. In patients with CF, lack of CF transmembrane …

CFTR, mucins, and mucus obstruction in cystic fibrosis

SM Kreda, CW Davis, MC Rose - Cold Spring …, 2012 - perspectivesinmedicine.cshlp.org
Mucus pathology in cystic fibrosis (CF) has been known for as long as the disease has been
recognized and is sometimes called mucoviscidosis. The disease is marked by mucus …

[HTML][HTML] The role of airway epithelium and blood neutrophils in the inflammatory response in cystic fibrosis

SWJ Terheggen-Lagro, GT Rijkers… - Journal of Cystic …, 2005 - Elsevier
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) gene, which accounts for the cAMP-modulated chloride conductance of airway …

Effects of airway surface liquid pH on host defense in cystic fibrosis

AR Berkebile, PB McCray Jr - The international journal of biochemistry & …, 2014 - Elsevier
Cystic fibrosis is a lethal genetic disorder characterized by viscous mucus and bacterial
colonization of the airways. Airway surface liquid represents a first line of pulmonary …

Mucociliary clearance as an outcome measure for cystic fibrosis clinical research

SH Donaldson, TE Corcoran, BL Laube… - Proceedings of the …, 2007 - atsjournals.org
Current concepts of cystic fibrosis (CF) pathophysiology link ion transport abnormalities to
reduced airway surface liquid (ASL) hydration and impaired mucus clearance. It is likely that …