Cystic fibrosis (CF) lung disease is characterized by early and persistent mucus
accumulation and neutrophilic inflammation in the distal airways. Identification of the factors …

Cystic fibrosis (CF) lung disease reflects persistent bacterial infection of airway lumens.
Several hypotheses have been advanced to link mutations in the CFTR gene to the failure of …

Studies of the pathogenesis of cystic fibrosis (CF) and primary ciliary dyskinesia (PCD), as
well as novel genetic mouse models, vividly illustrate that effective “mucus clearance” is a …

Pseudomonas aeruginosa is a remarkably versatile environmental bacterium with an
extraordinary capacity to infect the cystic fibrosis (CF) lung. Infection with P. aeruginosa …

Rationale: Enhancing non–CFTR (cystic fibrosis transmembrane conductance regulator)-
mediated anion secretion is an attractive therapeutic approach for the treatment of cystic …

Background Whereas cilia damage and reduced cilia beat frequency have been implicated
as causative of reduced mucociliary clearance in smokers, theoretically mucociliary …

Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway
secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the …

Rationale: Chronic bronchitis (CB) is characterized by persistent cough and sputum
production. Studies were performed to test whether mucus hyperconcentration and …

Since the detection of the underlying gene defect, our knowledge of how the genetic
mutations in cystic fibrosis cause lung disease has increased substantially, but we still lack a …

All cells release nucleotides and are in one way or another involved in local autocrine and
paracrine regulation of organ function via stimulation of purinergic receptors. Significant …