Cystic fibrosis: basic chemical and cellular mechanisms.

Cystic fibrosis (CF) primarily affects epithelial-lined organs, the most important being the lungs. Much of the known pathogenesis of the disease has been elucidated by the identification of the gene mutated in CF which encodes the cystic fibrosis transmembrane conductance regulator, a chloride channel in the apical membrane of epithelial cells of the respiratory and intestinal tracts.