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)

doi:10.1016/j.jhep.2014.02.006

Characterization of animal models for primary sclerosing cholangitis (PSC)

J Hepatol. 2014 Jun;60(6):1290-303. doi: 10.1016/j.jhep.2014.02.006. Epub 2014 Feb 19.

Authors

Peter Fickert¹, Marion J Pollheimer², Ulrich Beuers³, Carolin Lackner⁴, Gideon Hirschfield⁵, Chantal Housset⁶, Verena Keitel⁷, Christoph Schramm⁸, Hanns-Ulrich Marschall⁹, Tom H Karlsen¹⁰, Espen Melum¹¹, Arthur Kaser¹², Bertus Eksteen¹³, Mario Strazzabosco¹⁴, Michael Manns¹⁵, Michael Trauner¹⁶; International PSC Study Group (IPSCSG)

Affiliations

¹ Research Unit for Experimental and Molecular Hepatology, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Austria; Institute of Pathology, Medical University of Graz, Austria. Electronic address: [email protected].
² Research Unit for Experimental and Molecular Hepatology, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Austria; Institute of Pathology, Medical University of Graz, Austria.
³ Tytgat Institute for Liver and Intestinal Research, Academic Medical Centre, University of Amsterdam, The Netherlands.
⁴ Institute of Pathology, Medical University of Graz, Austria.
⁵ Centre for Liver Research, Institute of Biomedical Research, School of Immunity and Infection, University of Birmingham, UK.
⁶ UPMC Univ Paris 06 & INSERM, UMR-S 938, Centre de Recherche Saint-Antoine, F-75012 Paris, France.
⁷ Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University Düsseldorf, Germany.
⁸ Medical University Hamburg-Eppendorf, Germany.
⁹ Wallenberg Laboratory, Department of Molecular and Clinical Medicine, University of Gothenburg, The Sahlgrenska Academy, Sweden.
¹⁰ Division of Gastroenterology and Hepatology, Department of Medicine, Rikshospitalet, Oslo, Norway; Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Research Institute of Internal Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Division of Gastroenterology, Department of Clinical Medicine, University of Bergen, Bergen, Norway.
¹¹ Division of Gastroenterology and Hepatology, Department of Medicine, Rikshospitalet, Oslo, Norway; Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Research Institute of Internal Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
¹² Division of Gastroenterology and Hepatology, Department of Medicine, University of Cambridge, Addenbrooek's Hospital, UK.
¹³ Centre for Liver Research, MRC Centre for Immune Regulation, Institute for Biomedical Research, Medical School, University of Birmingham, and The Queen Elizabeth Hospital, University Hospitals Birmingham NHS Trust, Birmingham, UK.
¹⁴ Section of Gastroenterology, University of Milan-Bicocca, Milan, Italy; Liver Center, Yale University School of Medicine, United States.
¹⁵ Division of Gastroenterology, Hepatology and Endocrinology, Medical University Hannover, Germany.
¹⁶ Hans Popper Laboratory of Molecular Hepatology, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Austria. Electronic address: [email protected].

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.

Keywords: Animal model; Bile acids; Biliary fibrosis; Cholangiopathies; Cholestatic liver disease; Primary sclerosing cholangitis.

Publication types

Review

MeSH terms

Animals
Cholangitis, Sclerosing* / pathology
Cholangitis, Sclerosing* / physiopathology
Cholangitis, Sclerosing* / therapy
Disease Models, Animal*
Humans

Abstract

Publication types

MeSH terms

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