title: Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology.
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.
authors.affiliation: 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.
authors.forename: Alicia K
authors.initials: AK
authors.lastname: Olivier
authors.affiliation: 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.
authors.forename: Katherine N
authors.initials: KN
authors.lastname: Gibson-Corley
authors.affiliation: 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 david-meyerholz@uiowa.edu.
authors.forename: David K
authors.initials: DK
authors.lastname: Meyerholz
mesh_terms: D000818:Animals; D001660:Biliary Tract Diseases / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology*; D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism; D004195:Disease Models, Animal; D005767:Gastrointestinal Diseases / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology*; D020022:Genetic Predisposition to Disease; D006801:Humans; D008107:Liver Diseases / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology*; D018181:Mice, Inbred CFTR; D009154:Mutation; D010182:Pancreatic Diseases / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology*; D010641:Phenotype; D013045:Species Specificity
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: cystic fibrosis; cystic fibrosis transmembrane conductance regulator; gastrointestinal tract; liver; pancreas