Cystic fibrosis (CF) is a common autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). CF affects multiple organs, including lungs, pancreas, intestine, liver, sweat glands, gallbladder and the male genital tract. Airway infection and inflammation currently cause most of the morbidity and mortality. Although several therapies have improved the lives of patients, current treatments are inadequate and CF remains a lethal disease. Our knowledge about the pathogenesis of the disease, its progression, and the state of the neonatal lung is inadequate. These gaps in our knowledge have hindered attempts to develop better treatments and preventions for CF lung disease. A major impediment to addressing these issues has been limitations of current animal models. Although mouse strains carrying null and missense CFTR mutations have made enormous contributions, CF mice do not develop the airway or pancreatic disease typically found in humans. We recently generated CF pigs that replicate many of the key features of human CF disease including intestinal obstruction, exocrine pancreatic destruction, micro-gallbladder, vas deferens abnormalities, focal billary cirrhosis, congenital airway structural abnormalities, and airway and sinus infection with time. The goals ofthe Animal Core will be to (1) Provide Program investigators with CF and non-CF pigs;(2) Help Program investigators successfully accomplish the aims of their Projects. The Animal Core will function seamlessly through already established interactions with the Project Leaders, Morphology Core, Imaging Core, and the Administrative Core. The success ofthe Animal Models Core is ensured because ofthe commitment, experience, and expertise that the personnel bring to the Core.
Cystic fibrosis is a corhmon life-shortening genetic disease that causes progressive lung failure due to recurrent infections and chronic inflammation. These studies will use the new cystic fibrosis pig model to better understand the early events in cystic fibrosis lung disease, thereby leading to better therapies.
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