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

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


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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

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


  • Cystic Fibrosis Transmembrane Conductance Regulator