Pseudomonas aeruginosa triggers CFTR-mediated airway surface liquid secretion in swine trachea.

ABSTRACT: Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the gene encoding for the anion channel cystic fibrosis transmembrane conductance regulator (CFTR). Several organs are affected in CF, but most of the morbidity and mortality comes from lung disease. Recent data show that the initial consequence of CFTR mutation is the failure to eradicate bacteria before the development of inflammation and airway remodeling. Bacterial clearance depends on a layer of airway surface liquid (ASL) consisting of both a mucus layer that traps, kills, and inactivates bacteria and a periciliary liquid layer that keeps the mucus at an optimum distance from the underlying epithelia, to maximize ciliary motility and clearance of bacteria. The airways in CF patients and animal mode

SUBMITTER: Luan X 

PROVIDER: S-EPMC4156708 | biostudies-literature | 2014 Sep

REPOSITORIES: biostudies-literature