The May issue of Nature Medicine unveils a new mouse model of cystic fibrosis (CF), revealing a key aspect of the disease's mechanism.
CF is a lethal disease in which a genetic mutation causes the lungs to clog with mucus. In humans, the mutation blocks chloride channels and increases sodium channels, but the same mutation in mice does not cause CF-like lung disease.
Richard Boucher and colleagues developed their model of lung disease using genetic manipulation to directly increase sodium channels in the lungs of mice. The resulting increase in salt concentration led to the decreased airway hydration and mucus plugs typically seen in CF patients.
The mouse model is the first to show that having enough liquid on airway surfaces is the key to preventing mucus buildup in the lungs. The model will be useful in testing whether therapies that improve airway hydration will be effective in CF, chronic bronchitis and other lung diseases.
Richard C. Boucher
University of North Carolina at Chapel Hill
Tel: +1 919 966 1077
Also available online.
(C) Nature Medicine press release,
Message posted by: Trevor M. D'Souza