PerspectiveCystic Fibrosis

The Development of Lung Disease in Cystic Fibrosis Pigs

Science Translational Medicine  28 Apr 2010:
Vol. 2, Issue 29, pp. 29ps20
DOI: 10.1126/scitranslmed.3001130

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Abstract

The leading cause of death in cystic fibrosis (CF) patients is lung disease; yet despite enormous research efforts, its pathogenesis is not well understood. Because CF mice do not develop human-like airway disease, CF pigs were produced on the premise that they would offer insights into the pathogenesis of CF lung disease. But CF pigs develop severe intestinal blockage and often die shortly after birth. Now, a team of scientists report results from five CF pigs that lived for ~2 to 6 months; during this time, they developed some of the key features of human CF lung disease and revealed that infection precedes inflammation.

Footnotes

  • Citation: J. J. Wine, The development of lung disease in cystic fibrosis pigs. Sci. Transl. Med. 2, 29ps20 (2010).

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