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 ofthe key features of human CF disease including intestinal obstruction, exocrine pancreatic destruction, micro-gallbladder, vas deferens abnormalities, focal biliary cirrhosis, congenital airway structural abnormalities, and airway and sinus infection with time. The Imaging Core has two main objectives: (1) Provide scientific support, expertise, and resources for acquisition of image datasets and subsequent analysis;and (2) Help Project Leaders successfully accomplish the aims of their Projects. State of- art image acquisition technology and analysis tools are readily available at the University of lowa for these Projects. The Imaging Core will function seamlessly through already established interactions with the Project Leaders, Morphology Core Director, the Translational Lung Imaging Research Program, and the lowa Comprehensive Lung Imaging Center.

Public Health Relevance

Cystic fibrosis is a common 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 and potentially new image-based assessments of disease activity.

Agency
National Institute of Health (NIH)
Type
Research Program Projects (P01)
Project #
5P01HL091842-07
Application #
8737941
Study Section
Heart, Lung, and Blood Program Project Review Committee (HLBP)
Project Start
Project End
Budget Start
Budget End
Support Year
7
Fiscal Year
2014
Total Cost
Indirect Cost
Name
University of Iowa
Department
Type
DUNS #
City
Iowa City
State
IA
Country
United States
Zip Code
52242
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