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Am J Physiol Gastrointest Liver Physiol. 2015 Mar 15;308(6):G459-71. doi: 10.1152/ajpgi.00146.2014. Epub 2015 Jan 15.

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

Author information

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.
2
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.

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.

KEYWORDS:

cystic fibrosis; cystic fibrosis transmembrane conductance regulator; gastrointestinal tract; liver; pancreas

PMID:
25591863
PMCID:
PMC4360044
DOI:
10.1152/ajpgi.00146.2014
[Indexed for MEDLINE]
Free PMC Article

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