While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is
now recognized as a single disease whose diverse symptoms stem from the wide tissue …

Pulmonary defense mechanisms preventing chronic bacterial infection. The lung is depicted
as an inverted funnel, reflecting the relative surface area of distal versus proximal airways …

Current theories of CF pathogenesis predict different predisposing “local environmental”
conditions and sites of bacterial infection within CF airways. Here we show that, in CF …

Gene therapy for cystic fibrosis (CF) lung disease requires efficient gene transfer to airway
epithelial cells after intralumenal delivery. Most gene transfer vectors so far tested have not …

Recent data indicate that cystic fibrosis (CF) airway mucus is anaerobic. This suggests that
Pseudomonas aeruginosa infection in CF reflects biofilm formation and persistence in an …

Pseudomonas aeruginosa is the major pathogen in the cystic fibrosis (CF) lung. Prevalence
is high and, once acquired, chronic infection will almost always ensue. Several hypotheses …

Epithelial tight junctions (TJs) provide an important route for passive electrolyte transport
across airway epithelium and provide a barrier to the migration of toxic materials from the …

The pathogenesis of cystic fibrosis (CF) lung disease is reviewed, focusing on an overview
of the physiologic mechanisms that regulate mucus transport. A major emphasis is placed …

The epithelial sodium channel (ENaC) constitutes the rate-limiting step for sodium
absorption across airway epithelia, which in turn regulates airway surface liquid (ASL) …

Cystic fibrosis (CF) lung disease is characterized by a self-perpetuating cycle of airway
obstruction, chronic bacterial infection, and vigorous inflammation that results in structural …