The development of lung disease in cystic fibrosis pigs

JJ Wine - Science translational medicine, 2010 - science.org
Science translational medicine, 2010science.org
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 …
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.
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