Salt and fluid absorption and secretion are two processes that are fundamental to epithelial
function and whole body fluid homeostasis, and as such are tightly regulated in epithelial …

The small airways of the human lung undergo pathological changes in pulmonary disorders,
such as chronic obstructive pulmonary disease (COPD), asthma, bronchiolitis obliterans and …

Pulmonary fibrosis and architectural remodeling of tissues can severely disrupt lung
function, often with fatal consequences. The etiology of pulmonary fibrotic diseases is varied …

Rationale: Airway remodeling in asthma is associated with the accumulation of fibroblasts,
the primary cell responsible for synthesis and secretion of extracellular matrix proteins. The …

Cystic fibrosis is a genetic disease caused by mutations in the CFTR gene, whereas chronic
obstructive pulmonary disease (COPD) is mainly caused by environmental factors (mostly …

The inflammatory airway disease cystic fibrosis (CF) is characterized by airway obstruction
due to mucus hypersecretion, airway plugging, and bronchoconstriction. The cystic fibrosis …

The ability to replace defective cells in an airway with cells that can engraft, integrate, and
restore a functional epithelium could potentially cure a number of lung diseases. Progress …

One of the key features associated with the substantial increase in life expectancy for
individuals with CF is an elevated predisposition to cancer, firmly established by recent …

Mutations in CFTR lead to dysfunction of tubular organs, which is currently attributed to
impairment of its conductive properties. We now show that CFTR regulates tight junction …

The arrival of cystic fibrosis transmembrane conductance regulator (CFTR) modulators as a
new class of treatment for cystic fibrosis (CF) in 2012 represented a pivotal advance in …