Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that
regulates the transport of ions and the movement of water across the epithelial barrier …

Cystic fibrosis (CF), the most common fatal monogenic disease in the United States, results
from mutations in CF transmembrane conductance regulator (CFTR), a chloride channel …

Loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator
(CFTR) compromise epithelial HCO3− and Cl− secretion, reduce airway surface liquid pH …

Lung disease is the major cause of death in cystic fibrosis (CF), but there is no evidence for
overt lung involvement at birth. We show here that the same is true for the gene targeted cftr …

Cystic fibrosis (CF) is a multisystem autosomal recessive disorder caused by mutations of
the cystic fibrosis transmembrane regulator (CFTR), a protein that regulates cyclic-AMP …

Cystic fibrosis (CF) patients are hypersusceptible to chronic Pseudomonas aeruginosa lung
infections. Cultured human airway epithelial cells expressing the ΔF508 allele of the cystic …

Cystic fibrosis (CF), the most common lethal single-gene disorder affecting Northern
Europeans and North Americans, is caused by mutations in the cystic fibrosis …

The leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease.
Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as …

The most common mutation in cystic fibrosis, ΔF508, results in a cystic fibrosis
transmembrane conductance regulator (CFTR) protein that is retained in the endoplasmic …

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) protein1. While 70% of CF chromosomes carry a deletion of the phenylalanine …