Airway disease currently causes most of the morbidity and mortality in patients with cystic
fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing …

Although destructive airway disease is evident in young children with cystic fibrosis (CF),
little is known about the nature of the early CF lung environment triggering the disease. To …

Airway mucus is a complex mixture of secretory products that provides a multifaceted defense
against pulmonary infection. Mucus contains antimicrobial peptides (eg, defensins) and …

Rationale: Electronic cigarette (e-cig) use has been widely adopted under the perception of
safety. However, possibly adverse effects of e-cig vapor in never-smokers are not well …

Epithelial sodium channel (ENaC) blockers have been proposed as a therapy to restore
mucus clearance (MC) in cystic fibrosis (CF) airways. The therapeutic effects of the first …

Inhibition of airway epithelial sodium channel (ENaC) function enhances mucociliary clearance
(MCC). ENaC is positively regulated by channel-activating proteases (CAPs), and CAP …

10.1164/rccm.200406-735OC Florida red tide brevetoxins are sodium channel neurotoxins
produced by the dinoflagellate Karenia brevis. When aerosolized, the toxin causes airway …

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

Antigen challenge can elicit an allergic inflammatory response in the airways that involves
eosinophils, basophils, and neutrophils and that is expressed physiologically as a late airway …

Amiloride improves mucociliary clearance (MC) by blocking airway epithelial sodium channels
(ENaC) and expanding airway surface liquid (ASL). However, the low potency and rapid …