Cystic fibrosis, a multi-organ genetic disease, is characterized by abnormal function of the
cystic fibrosis transmembrane conductance regulator (CFTR) protein, a chloride channel at …

Although cystic fibrosis (CF) patients exhibit signs of endothelial perturbation, the functions
of the cystic fibrosis conductance regulator (CFTR) in vascular endothelial cells (EC) are …

Cystic fibrosis transmembrane conductance regulator (CFTR), a Cl−-selective ion channel,
is a prototypic member of the ATP-binding cassette transporter superfamily that is expressed …

The gene that encodes for the cystic fibrosis transmembrane regulator protein (CFTR) was
identified in 1989, yet major pathophysiologic questions remain unanswered. There is …

Studies of the pathogenesis of cystic fibrosis (CF) and primary ciliary dyskinesia (PCD), as
well as novel genetic mouse models, vividly illustrate that effective “mucus clearance” is a …

Method: MIP scores were established using a Canadian CF population (n= 2,492) as
estimates of the proportion of patients with MI among all patients carrying the same CFTR …

We have demonstrated previously the regulation of Cl−/HCO 3− exchange activity by the
cystic fibrosis transmembrane conductance regulator (CFTR) in model systems of cells …

Submucosal glands of the tracheobronchial airways provide the important functions of
secreting mucins, antimicrobial substances, and fluid. This review focuses on the ionic …

Cystic fibrosis (CF) is caused by mutations in the gene coding for the CF transmembrane
conductance regulator (CFTR). From human normal tracheal submucosal gland cells in …

Rationale: In cystic fibrosis (CF) a reduction in airway surface liquid (ASL) height
compromises mucociliary clearance, favoring mucus plugging and chronic bacterial …