Abstract NEDD4-2 (also known as NEDD4L, neural precursor cell expressed
developmentally down-regulated 4-like) is a ubiquitin protein ligase of the Nedd4 family …

Rationale: The respiratory tract is constantly exposed to airborne microorganisms.
Nevertheless, normal airways remain sterile without recruiting phagocytes. This innate …

Background: The loss of cystic fibrosis transmembrane conductance regulator (CFTR)
mediated chloride conductance does not fully explain the diverse pathologies evident in …

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) protein, an epithelial chloride channel expressed in the airways, pancreas, testis …

Animal models for cystic fibrosis (CF) have contributed significantly to our understanding of
disease pathogenesis. Here we describe development and characterization of the first cystic …

Here we report the effects of gentamicin treatment on cystic fibrosis transmembrane
regulator (CFTR) production and function in CF airway cells and patients with CF with …

Drug discovery by high-throughput screening is a promising approach to develop new
therapies for the most common lethal genetic disease, cystic fibrosis. Because disease …

It is increasingly being recognized that cells coordinate the activity of separate ion channels
that allow electrolytes into the cell. However, a perplexing problem in channel regulation has …

The concentration of salt in the thin layer of fluid at the surface of large airways, the airway-
surface liquid (ASL), is believed to be of central importance in airway physiology and in the …

The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a cAMP-
regulated epithelial Cl− channel that, when defective, causes cystic fibrosis. Screening of a …