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 …

Somatic cell gene targeting combined with nuclear transfer cloning presents tremendous
potential for the creation of new, large-animal models of human diseases. Mouse disease …

Although cystic fibrosis (CF), an autosomal recessive disease caused by mutations in the
gene encoding for the CF transmembrane conductance regulator (CFTR), seems a good …

Airway submucosal glands contribute to airway surface liquid (ASL) composition and
volume, both important for lung mucociliary clearance. Serous acini generate most of the …

The mucosa of the proximal airways defends itself and the lower airways from inhaled
irritants such as capsaicinoids, allergens, and infections by several mechanisms. Sensory …

Noncholinergic neurons contribute to innate airway defenses by releasing vasoactive
intestinal peptides (VIP), which stimulates the submucosal glands to produce a bicarbonate …

Background: Although cystic fibrosis (CF) seems a good candidate for gene therapy (GT), 15
years of intense investigations and a number of clinical trials have not yet produced a viable …

Trefoil factors are small, protease-resistant proteins first discovered in the gastrointestinal
tract and found to be important in restitution and repair after epithelial loss. This has been …

Airway submucosal gland serous acinar cells are crucial to the secretion of airway surface
liquid in cartilaginous airways. However, the molecular mechanisms of serous cell ion and …

Desconhece-se, até o momento, o efeito funcional de medicamentos anticolinérgicos de
longa ação, em pacientes com fibrose cística (FC), apesar do seu uso habitual, com …