Characterization of animal models for primary sclerosing cholangitis (PSC)

Peter Fickert, Marion J Pollheimer, Ulrich Beuers, Carolin Lackner, Gideon Hirschfield, Chantal Housset, Verena Keitel, Christoph Schramm, Hanns-Ulrich Marschall, Tom H Karlsen, Espen Melum, Arthur Kaser, Bertus Eksteen, Mario Strazzabosco, Michael Manns, Michael Trauner, International PSC Study Group (IPSCSG)

Research output: Contribution to journalReview articlepeer-review

79 Citations (Scopus)
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Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholangiopathy characterized by biliary fibrosis, development of cholestasis and end stage liver disease, high risk of malignancy, and frequent need for liver transplantation. The poor understanding of its pathogenesis is also reflected in the lack of effective medical treatment. Well-characterized animal models are utterly needed to develop novel pathogenetic concepts and study new treatment strategies. Currently there is no consensus on how to evaluate and characterize potential PSC models, which makes direct comparison of experimental results and effective exchange of study material between research groups difficult. The International Primary Sclerosing Cholangitis Study Group (IPSCSG) has therefore summarized these key issues in a position paper proposing standard requirements for the study of animal models of PSC.

Original languageEnglish
Pages (from-to)1290-303
Number of pages14
JournalJournal of Hepatology
Volume60
Issue number6
Early online date19 Feb 2014
DOIs
Publication statusPublished - 1 Jun 2014

Keywords

  • Animals
  • Cholangitis, Sclerosing
  • Disease Models, Animal
  • Humans
  • Animal model
  • Biliary fibrosis
  • Bile acids
  • Cholangiopathies
  • Cholestatic liver disease
  • Primary sclerosing cholangitis

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