Affiliation:
1. Department of Pathology and Center for Gene Therapy of Cystic Fibrosis and Other Genetic Diseases, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa
Abstract
Multiple organ systems, including the gastrointestinal tract, pancreas, and hepatobiliary systems, are affected by cystic fibrosis (CF). Many of these changes begin early in life and are difficult to study in young CF patients. Recent development of novel CF animal models has expanded opportunities in the field to better understand CF pathogenesis and evaluate traditional and innovative therapeutics. In this review, we discuss manifestations of CF disease in gastrointestinal, pancreatic, and hepatobiliary systems of humans and animal models. We also compare the similarities and limitations of animal models and discuss future directions for modeling CF.
Funder
HHS | NIH | National Heart, Lung, and Blood Institute (NHBLI)
HHS | NIH | National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Cystic Fibrosis Foundation (CFF)
Publisher
American Physiological Society
Subject
Physiology (medical),Gastroenterology,Hepatology,Physiology
Cited by
42 articles.
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