Mucus pathology in cystic fibrosis (CF) has been known for as long as the disease has been
recognized and is sometimes called mucoviscidosis. The disease is marked by mucus …

Airway disease currently causes most of the morbidity and mortality in patients with cystic
fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing …

Alveolar epithelial type II (ATII) cells are small, cuboidal cells that constitute≈ 60% of the
pulmonary alveolar epithelium. These cells are crucial for repair of the injured alveolus by …

The lung surface of air‐breathing vertebrates is formed by a continuous epithelium that is
covered by a fluid layer. In the airways, this epithelium is largely pseudostratified consisting …

Despite extensive research into the pathogenesis of acute lung injury and the acute
respiratory distress syndrome (ALI/ARDS), mortality remains high at approximately 40 …

The resolution of alveolar edema is regulated by active sodium and chloride transport
across the pulmonary epithelium, including alveolar epithelial type I and II cells as well as …

Efficient gas exchange in the lungs depends on regulation of the amount of fluid in the thin
(average 0.2 μm) liquid layer lining the alveolar epithelium. Fluid fluxes are regulated by ion …

Maintenance of normal epithelial ion and water transport in the lungs includes providing a
thin layer of surface liquid that coats the conducting airways. This airway surface liquid is …

Although lung-protective ventilation strategies have substantially reduced mortality of acute
lung injury patients there is still a need for new therapies that can further decrease mortality …

Previous studies in intact lung suggest that CFTR may play a role in cAMP-regulated fluid
transport from the distal air spaces of the lung. However, the potential contribution of …