Aided by a new experimental model, scientists are a step closer to understanding how cystic fibrosis (CF) causes lung disease in people with the condition. The findings, published online April 28 in the journal Science Translational Medicine, could help improve treatments for lung disease, which causes most of the deaths and disability among people with cystic fibrosis.
In particular, the study by a University of Iowa research team and colleagues at the University of Missouri appears to answer a long-standing “chicken and egg” question about which comes first in cystic fibrosis lungs — infection or inflammation.
The new experimental model that Stoltz and his colleagues used was pigs with a cystic fibrosis-causing gene mutation. The team generated the pig model in the hope that it would more closely mimic the human disease than mouse models do.
By studying the CF pigs through their first six months of life, the team has shown that these animals do develop lung disease typical of what is seen in humans, including infection in the lungs, inflammation, and accumulation of mucus in the airways, which is a significant problem for patients with cystic fibrosis.
“This is a really great example where the pig serves as a model for what happens in the human, and the pig reacts to this disease in nearly the same way,” says Randall Prather, distinguished professor of reproductive biotechnology at MU. “In contrast, when you use mice, they don’t get the lung disease that is common in patients with cystic fibrosis.”
The team also found that the lungs of newborn CF pigs tended to be infected with more bacteria than lungs of control pigs.