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Scientists Create Pigs That Can Develop Cystic Fibrosis

September 26, 2008

Scientists hope that a new development, which allows them to create pigs that develop cystic fibrosis in the same way the people do, will give them new insight into fighting the disease.

Scientists at the University of Iowa and the University of Missouri have created genetically engineered piglets with the same mutation that causes cystic fibrosis in humans, they report in the journal Science.

Up to this point, scientists have been unable to find any hints that may lead to a possible cure, but this latest development should offer an unprecedented look at the disease, said Dr. Michael Welsh of the University of Iowa.

CF is caused when people inherit two mutated copies of a gene called CTFR. The disease causes mucus to accumulate and clog some of the organs in the body, especially the lungs and pancreas.

About 70,000 people worldwide, including 30,000 people in the United States, have CF. People with the disease can expect to live to about age 37, according to the Cystic Fibrosis Foundation, which helped fund the study.

Scientists create animal “models” of a disease in order to perform experiments that would not be possible with people. Mice with the genetic abnormality that causes cystic fibrosis have been developed, but the disease presents itself very differently in these rodents than in people.

“Unfortunately, the mice leave something to be desired,” said Welsh, who helped lead the study.

“They don’t get the pancreatic disease like people with CF get. They don’t get the lung disease like people with CF get. They don’t get the intestinal disease like people with CF get. There’s been many questions that can’t be answered,” he added.

Christopher Penland of the foundation admitted there is some difficulty in pinpointing the onset of the disease.
This is the latest example of pigs used for human medical needs.

The scientists developed pigs with cystic fibrosis because their lungs have many characteristics of human lungs.

“Right now, if you want to do experiments to find treatments or therapies for the lung disease that is fatal for people with CF, you would have to experiment on kids that have CF,” Randy Prather of the University of Missouri added.

Now the developments leading to the disease in pigs allows scientists to “start experimenting in ways that have never been possible,” Prather said in a statement.

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