We have tested two hypotheses: 1) the cystic fibrosis transmembrane conductance regulator
(CFTR) represents the predominant Cl conductance in the apical membrane of human …

A major limitation in the study of vectorial ion transport, secretion, and differentiated function
in the human airway epithelium has been the lack of suitable cell culture systems. Progress …

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 …

The cystic fibrosis (CF) gene codes for CF transmembrane regulator (CFTR), a small-
conductance linear Cl-channel, but numerous studies have identified a larger conductance …

Cystic fibrosis (CF) is a mucoobstructive pathology associated with chronic inflammation and
chronic bacterial infection of the lungs. Mutations in the CF gene lead to dysfunction of the …

Inhibitors of guanosine 3′, 5′-cyclic monophosphate (cGMP)-inhibited
phosphodiesterases stimulate Cl− transport across the nasal epithelia of cystic fibrosis mice …

1. An improved novel plasmid backbone, pTrial10, has been developed. We have used this
vector to deliver the cDNA for the cystic fibrosis transmembrane conductance regulator …

In 1983, Paul Quinton showed that the chloride permeability of sweat duct epithelium was
essentially abolished in cystic fibrosis (CF)(1). Shortly after, cyclic adenosine …

Using cell culture models, we have investigated the relative importance of cystic fibrosis
transmembrane conductance regulator (CFTR) and calcium-activated chloride channels …

Human airway serous cells secrete antibiotic-rich fluid, but, in cystic fibrosis (CF), Cl−-
dependent fluid secretion is impaired by defects in CF transmembrane conductance …