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Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology
Journal article   Open access   Peer reviewed

Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology

Alicia K Olivier, Katherine N Gibson-Corley and David K Meyerholz
American journal of physiology: Gastrointestinal and liver physiology, Vol.308(6), pp.G459-G471
03/15/2015
DOI: 10.1152/ajpgi.00146.2014
PMCID: PMC4360044
PMID: 25591863
url
https://doi.org/10.1152/ajpgi.00146.2014View
Published (Version of record) Open Access

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.
Gastrointestinal Diseases - physiopathology Species Specificity Cystic Fibrosis - physiopathology Humans Gastrointestinal Diseases - etiology Liver Diseases - etiology Biliary Tract Diseases - physiopathology Pancreatic Diseases - etiology Pancreatic Diseases - physiopathology Disease Models, Animal Mice, Inbred CFTR Genetic Predisposition to Disease Pancreatic Diseases - metabolism Cystic Fibrosis - metabolism Cystic Fibrosis Transmembrane Conductance Regulator - metabolism Cystic Fibrosis - complications Phenotype Animals Gastrointestinal Diseases - metabolism Cystic Fibrosis - genetics Biliary Tract Diseases - etiology Cystic Fibrosis Transmembrane Conductance Regulator - genetics Liver Diseases - physiopathology Mutation Liver Diseases - metabolism Biliary Tract Diseases - metabolism

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