NEW PIG MODEL OF CYSTIC FIBROSIS LAYS GROUNDWORK FOR BETTER UNDERSTANDING OF HUMAN DISEASE
The study, published in the Sept. 26 edition of “Science,” was funded in part by the National Heart, Lung, and Blood Institute (NHLBI), along with the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), both of the National Institutes of Health, as well as the Cystic Fibrosis Foundation.
CF is an inherited disease of the mucus-secreting glands which is caused by mutations in the gene responsible for making the protein cystic fibrosis transmembrane conductance regulator (CFTR), important for making sweat, digestive juices, and mucus. CF affects multiple organs, including the lungs, pancreas, liver, intestines, sinuses, and sex organs. In CF, mucus becomes thick and sticky, and builds up in the lungs and in the pancreas, blocking the airways, and disrupting the digestive system, resulting in recurrent, destructive infections and trouble digesting food. Respiratory failure and liver disease are the most common causes of death in CF.
Before now, mice have been the only animal model for CF. However since mice do not exhibit typical symptoms of CF, and the lung and liver diseases found in humans, finding a better model was crucial to furthering CF research.
“This represents a significant advance in research on cystic fibrosis. Until now, no animal model has come close to mimicking the disease as seen in humans. This model offers unprecedented opportunities to understand how the respiratory disease develops during childhood which may lead to novel prevention and therapeutic strategies,” said Elizabeth G. Nabel, M.D., director, NHLBI.
“By tracking how the lungs of these pigs respond to challenges to their respiratory systems introduced by the environment, we hope to better understand how the complications of CF progress in children,” said Michael J. Welsh, M.D., University of Iowa and the Howard Hughes Medical Institute, and senior author of the study.
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