Restoring Airway Surface Liquid in Cystic Fibrosis

The current pathophysiological model of cystic fibrosis lung disease assumes that defective expression, trafficking, or function of the cystic fibrosis transmembrane regulator (CFTR) protein leads to impaired epithelial chloride secretion and sodium hyperabsorption. This process, in turn, results in the depletion of airway surface liquid and abnormal mucociliary transport. Retention of mucus is thought to favor bacterial overgrowth, which then triggers a cycle of repeated or chronic infections associated with intense neutrophilic airway inflammation.Defective mucociliary transport itself can cause airway inflammation, as has been shown in mice that have an overexpression of the beta subunit of the epithelial sodium . . .