FRIDAY, Sept. 26 (HealthDay News) -- Researchers say they have developed a model for cystic fibrosis (CF) in piglets that may help improve understanding of the disease in human infants and help in the discovery of new treatments.
The finding, by researchers at the University of Iowa and the University of Missouri, was published in the Sept. 26 issue of Science.
Cystic fibrosis, a common hereditary disease, affects multiple organ systems, including the intestines, pancreas and lungs. A primary cause of death and disability in these patients is lung disease. Researchers have previously studied mice with CF-causing mutations to study the disease, but the mice do not develop many of the typical symptoms that affect humans.
"Lack of a better model has hampered our ability to answer long-standing questions in CF," co-lead author Christopher Rogers, a former postdoctoral fellow in internal medicine at the University of Iowa College of Medicine, said in a university-issued news release. "The CF pig provides a unique opportunity to study one of the most common genetic diseases, and we hope to translate this new knowledge into better therapies and preventions."
To create the pigs with cystic fibrosis, the researchers used gene targeting to disrupt one copy of the normal CFTR gene in pig cells, then cloned these altered cells to produce pigs with only one good copy of the gene. The pigs were then bred naturally, and about one in four of the piglets was born with two bad copies of the gene -- the hallmark of cystic fibrosis.
"Researchers can now begin to study the disease progression as it is happening, something not possible in humans," co-lead author David Meyerholz, Iowa assistant professor of pathology, said in the news release.
The Cystic Fibrosis Foundation has more about cystic fibrosis and its treatment.