The development of lung disease in cystic fibrosis pigs

• Published in: Science translational medicine Vol. 2; no. 29; p. 29ps20
• Main Author: Wine, Jeffrey J
• Format: Journal Article
• Language: English
• Published: United States 28-04-2010
• Subjects: Animals; Cystic Fibrosis - pathology; Cystic Fibrosis Transmembrane Conductance Regulator - genetics; Cystic Fibrosis Transmembrane Conductance Regulator - metabolism; Disease Models, Animal; Humans; Lung - pathology; Mice; Swine
• ISSN: 1946-6242
• DOI: 10.1126/scitranslmed.3001130

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.