Airway surface dehydration in cystic fibrosis: pathogenesis and therapy

RC Boucher - Annu. Rev. Med., 2007 - annualreviews.org
Cystic fibrosis (CF) lung disease reflects the failure of airways defense against chronic
bacterial infection. Studies of CF cultures, transgenic mice, and CF patients suggest that the …

Evidence for airway surface dehydration as the initiating event in CF airway disease

RC Boucher - Journal of internal medicine, 2007 - Wiley Online Library
Cystic fibrosis (CF) lung disease reflects persistent bacterial infection of airway lumens.
Several hypotheses have been advanced to link mutations in the CFTR gene to the failure of …

Cystic fibrosis: a disease of vulnerability to airway surface dehydration

RC Boucher - Trends in molecular medicine, 2007 - cell.com
Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway
secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the …

Cystic fibrosis and other respiratory diseases of impaired mucus clearance

A Livraghi, SH Randell - Toxicologic pathology, 2007 - journals.sagepub.com
Exposed to a diverse array of potentially noxious agents, the respiratory tract is protected by
a highly developed innate defense system. Physiologically regulated epithelial ion and …

Sodium channels and cystic fibrosis

SH Donaldson, RC Boucher - Chest, 2007 - Elsevier
The epithelial sodium channel (ENaC) represents the rate-limiting step of sodium absorption
across airway epithelia and thereby constitutes the major pathway for volume absorption …

Coordinated release of nucleotides and mucin from human airway epithelial Calu‐3 cells

SM Kreda, SF Okada, CA Van Heusden… - The Journal of …, 2007 - Wiley Online Library
The efficiency of the mucociliary clearance (MCC) process that removes noxious materials
from airway surfaces depends on the balance between mucin secretion, airway surface …

Differential effects of cyclic and constant stress on ATP release and mucociliary transport by human airway epithelia

B Button, M Picher, RC Boucher - The Journal of physiology, 2007 - Wiley Online Library
In the lungs, the first line of defence against bacterial infection is the thin layer of airway
surface liquid (ASL) lining the airway surface. The superficial airway epithelium exhibits …

Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis

MD Amaral, K Kunzelmann - Trends in pharmacological sciences, 2007 - cell.com
One of the major challenges facing the pharmaceutical field is the identification of
novel,'druggable'targets common to distinct diseases that, despite their clinical diversity …

Cystic fibrosis: the mechanisms of pathogenesis of an inherited lung disorder

MT Clunes, RC Boucher - Drug Discovery Today: Disease Mechanisms, 2007 - Elsevier
Cystic fibrosis patients exhibit lung disease consistent with a failure of innate airway defense
mechanisms. The link between abnormal ion transport and disease initiation and …

Mechano‐sensitivity of epithelial sodium channels (ENaCs): laminar shear stress increases ion channel open probability

M Althaus, R Bogdan, WG Clauss… - The FASEB …, 2007 - Wiley Online Library
Epithelial cells are exposed to a variety of mechanical forces, but little is known about the
impact of these forces on epithelial ion channels. Here we show that mechanical activation …