PerspectiveMedicine

Letting go of mucus

Science  15 Aug 2014:
Vol. 345, Issue 6198, pp. 730-731
DOI: 10.1126/science.1258493

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Summary

The genetic disease cystic fibrosis (CF) results from mutations that disable an anion channel called cystic fibrosis transmembrane conductance regulator (CFTR). Loss of CFTR's anion channel functions triggers a cascade of defects that reduces the lung's innate mucosal defenses, including the critical component of mucus clearance (1, 2). The lungs of individuals with CF, which appear grossly normal at birth, quickly become infected with bacteria and fungi localized within the lung mucus, which, like mucus in other CF organs, is thicker and more difficult to clear (3). The pathogenesis of CF airways has been difficult to determine because appropriate animal models have been lacking. On page 818 in this issue, Hoegger et al. (4) find that lung disease in a piglet model of CF is caused by abnormal tethering of mucus to the submucosal glands that produce it.