Background The airways of patients with cystic fibrosis (CF) are highly complex, subject to
various environmental conditions as well as a distinct microbiota. Pseudomonas aeruginosa …

Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator
undermine many host defense systems by inhibiting the function of airway-surface liquid …

Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in a gene encoding
a protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). The CFTR …

Lung disease in people with cystic fibrosis (CF) is initiated by defective host defense that
predisposes airways to bacterial infection. Advanced CF is characterized by a deficit in …

Submucosal glands contribute to airway surface liquid (ASL), a film that protects all airway
surfaces. Glandular mucus comprises electrolytes, water, the gel-forming mucin MUC5B …

Mucus selectively controls the transport of molecules, particulate matter, and
microorganisms to the underlying epithelial layer. It may be desirable to weaken the mucus …

Gel-forming mucins, the primary macromolecular components of airway mucus, facilitate
airway clearance by mucociliary transport. In cystic fibrosis (CF) altered mucus properties …

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 …

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) protein, an epithelial chloride channel expressed in the airways, pancreas, testis …

Most airway mucus is produced by submucosal glands in response to neural signals. Gland
mucus traps microbes, inhibits their replication, and clears them from the airways. In cystic …