Cystic fibrosis (CF) is a common, life-threatening, multisystemic, autosomal recessive
disorder. In the last few years, giant steps have been made with regard to the understanding …

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 …

Update on pathogenesis of cystic fibrosis lung disease : Current Opinion in Pulmonary
Medicine Update on pathogenesis of cystic fibrosis lung disease : Current Opinion in …

Michael J. Welsh'and Alan E. Smith+'Howard Hughes Medical Institute Departments of
Internal Medicine and of Physiology and Biophysics University of Iowa College of Medicine …

Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator
undermine many host defense systems by inhibiting the function of airway-surface liquid …

Lung disease in cystic fibrosis is primarily due to a defect in the cystic fibrosis
transmembrane regulating protein (CFTR). This results in abnormal chloride transfer across …

Cystic fibrosis (CF) remains the most common life-shortening hereditary disease in white
populations, with high morbidity and mortality related to chronic airway mucus obstruction …

Although there has been impressive progress in the elucidation of the genetic and molecular
basis of cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The …

Cystic fibrosis (CF) was distinguished from celiac disease in 1938. Then, it was a pathologic
diagnosis, life expectancy was approximately 6 months, and the autosomal recessive …

Cystic fibrosis patients exhibit lung disease consistent with a failure of innate airway defense
mechanisms. The link between abnormal ion transport and disease initiation and …