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 …

Pilewski, Joseph M., and Raymond A. Frizzell. Role of CFTR in Airway Disease. Physiol.
Rev. 79, Suppl.: S215–S255, 1999.—Cystic fibrosis (CF) is caused by mutations in the gene …

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 …

▪ Abstract The developing distal lung epithelium displays an evolving liquid transport
phenotype, reflecting a changing and dynamic balance between Cl− ion secretion and Na+ …

Activation of the Ca2+ activated Cl− channel TMEM16A is proposed as a treatment in
inflammatory airway disease. It is assumed that activation of TMEM16A will induce …

Submucosal glands (SMGs) are a prominent structure that lines human cartilaginous
airways. Although it has been assumed that SMGs contribute to respiratory defense, that …

The tracheobronchial submucosal glands secrete liquid that is important for hydrating airway
surfaces, supporting mucociliary transport, and serving as a fluid matrix for numerous …

1 Epithelial Na+ channels (ENaCs) are inhibited by the cystic fibrosis transmembrane
conductance regulator (CFTR) when CFTR is activated by protein kinase A. Since cAMP …

The development of effective therapies for cystic fibrosis (CF) requires animal models that
can appropriately reproduce the human disease phenotype. CF mouse models have …

The combination of both Cl− and HCO 3− secretion inhibitors causes an accumulation of
mucins within the submucosal gland ducts of acetylcholine (ACh)-treated bronchi [SK Inglis …