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

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  • Author(s): Olivier AK;Olivier AK; Gibson-Corley KN; Gibson-Corley KN; Meyerholz DK; Meyerholz DK
  • Source:
    American journal of physiology. Gastrointestinal and liver physiology [Am J Physiol Gastrointest Liver Physiol] 2015 Mar 15; Vol. 308 (6), pp. G459-71. Date of Electronic Publication: 2015 Jan 15.
  • Publication Type:
    Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • Language:
    English
  • Additional Information
    • Source:
      Publisher: American Physiological Society Country of Publication: United States NLM ID: 100901227 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1522-1547 (Electronic) Linking ISSN: 01931857 NLM ISO Abbreviation: Am J Physiol Gastrointest Liver Physiol Subsets: MEDLINE
    • Publication Information:
      Original Publication: Bethesda, MD : American Physiological Society
    • Subject Terms:
      Biliary Tract Diseases/*physiopathology ; Cystic Fibrosis/*complications ; Gastrointestinal Diseases/*physiopathology ; Liver Diseases/*physiopathology ; Pancreatic Diseases/*physiopathology; Animals ; Biliary Tract Diseases/etiology ; Biliary Tract Diseases/metabolism ; 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 ; Genetic Predisposition to Disease ; Humans ; Liver Diseases/etiology ; Liver Diseases/metabolism ; Mice, Inbred CFTR ; Mutation ; Pancreatic Diseases/etiology ; Pancreatic Diseases/metabolism ; Phenotype ; Species Specificity
    • 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.
      (Copyright © 2015 the American Physiological Society.)
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    • Grant Information:
      P30 DK-54759 United States DK NIDDK NIH HHS; P01 HL091842 United States HL NHLBI NIH HHS; HL-51670 United States HL NHLBI NIH HHS; R24 DK096518 United States DK NIDDK NIH HHS; R24 DK-096518 United States DK NIDDK NIH HHS; HL-091842 United States HL NHLBI NIH HHS; P30 DK054759 United States DK NIDDK NIH HHS
    • Contributed Indexing:
      Keywords: cystic fibrosis; cystic fibrosis transmembrane conductance regulator; gastrointestinal tract; liver; pancreas
    • Accession Number:
      126880-72-6 (Cystic Fibrosis Transmembrane Conductance Regulator)
    • Publication Date:
      Date Created: 20150117 Date Completed: 20150512 Latest Revision: 20181113
    • Publication Date:
      20231215
    • Accession Number:
      PMC4360044
    • Accession Number:
      10.1152/ajpgi.00146.2014
    • Accession Number:
      25591863