Mucus and Airway Epithelium Alterations in Cystic Fibrosis

Cystic fibrosis (CF) is a multiorgan lethal inherited disease, affecting about 2000-5000 children and adults of Caucasian origin. Most of the major manifestations of the disease are related to a primary defect in the protein product of the cystic fibrosis transmembrane conductance regulator (CFTR) gene which is normally present in the apical membrane of the airway surface epithelium and submucosal glands [ 1 ]. The pulmonary complications, characterized by airway mucus accumulation, recurrent bacterial infections in the lower respiratory tract and bronchial obstruction are the major causes of the morbidity and mortality in CF disease [ 2 ]. These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.