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 productive 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 and the progression of the disease, as well as 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. CF pigs replicate many of the 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-the-art image acquisition technology and analysis tools are readily available at the University of Iowa for these Projects. The Imaging Core will function seamlessly through already established interactions with the Project Leaders, Core Directors, the Translational Lung Imaging Research Program, and the Iowa Comprehensive Lung Imaging Center.
CORE C ? IMAGING PROJECT NARRATIVE Cystic fibrosis is a common life-shortening disease that causes progressive lung failure due to recurrent infections and chronic inflammation. These studies will use novel animal models and advanced imaging modalities to understand normal lung defenses and the derangements produced by CFTR mutation at the onset and with progression of the disease. These studies will also help develop better therapies for cystic fibrosis lung disease.
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