The development of a successful gene therapy has many stages, including preclinical
testing in animal models and proof of principle clinical studies. A variety of diseases affect …

Mouse models for cystic fibrosis (CF) with no CFTR function (Cftr-/-) have the disadvantage
that most animals die of intestinal obstruction shortly after weaning. The objective of this …

Cystic fibrosis (CF) is a multisystemic, autosomal recessive disorder caused by mutations in
the CFTR (cystic fibrosis transmembrane conductance regulator) gene, with the majority of …

In the airway epithelia, extracellular adenosine modulates a number of biological processes.
However, little is known about adenosine's role in the inflammatory responses of airway …

—We investigated how cystic fibrosis (CF) alters the relationship between Cl− and mucin
secretion in cultures of non-CF and CF human tracheobronchial gland mucous (HTGM and …

The cloning of the defective gene in cystic fibrosis (CFTR) is the most important step to date
toward understanding the pathogenesis of the disease and developing novel therapeutic …

Because of its possible importance in cystic fibrosis (CF) pulmonary pathogenesis, the effect
of anion and liquid secretion inhibitors on airway mucociliary transport was examined. When …

Several recent reports have suggested that airway inflammation may precede infection and
relate to an endogenous dysregulation of pro-inflammatory cytokines in cystic fibrosis (CF) …

The intracellular localization of cystic fibrosis transmembrane conductance regulator (CFTR)
in native tissues is a major issue in the study of mutation, processing, and trafficking effects …

The genetic disorder cystic fibrosis (CF) is the most common lethal monogenic disease in
Caucasian populations, estimated to affect one out of 2500–4000 newborns. While CF …