title: Characterization of animal models for primary sclerosing cholangitis (PSC).
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.
authors.affiliation: 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: peter.fickert@medunigraz.at.
authors.forename: Peter
authors.initials: P
authors.lastname: Fickert
authors.affiliation: 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.
authors.forename: Marion J
authors.initials: MJ
authors.lastname: Pollheimer
authors.affiliation: Tytgat Institute for Liver and Intestinal Research, Academic Medical Centre, University of Amsterdam, The Netherlands.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Beuers
authors.affiliation: Institute of Pathology, Medical University of Graz, Austria.
authors.forename: Carolin
authors.initials: C
authors.lastname: Lackner
authors.affiliation: Centre for Liver Research, Institute of Biomedical Research, School of Immunity and Infection, University of Birmingham, UK.
authors.forename: Gideon
authors.initials: G
authors.lastname: Hirschfield
authors.affiliation: UPMC Univ Paris 06 & INSERM, UMR-S 938, Centre de Recherche Saint-Antoine, F-75012 Paris, France.
authors.forename: Chantal
authors.initials: C
authors.lastname: Housset
authors.affiliation: Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University Düsseldorf, Germany.
authors.forename: Verena
authors.initials: V
authors.lastname: Keitel
authors.affiliation: Medical University Hamburg-Eppendorf, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Schramm
authors.affiliation: Wallenberg Laboratory, Department of Molecular and Clinical Medicine, University of Gothenburg, The Sahlgrenska Academy, Sweden.
authors.forename: Hanns-Ulrich
authors.initials: HU
authors.lastname: Marschall
authors.affiliation: 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.
authors.forename: Tom H
authors.initials: TH
authors.lastname: Karlsen
authors.affiliation: 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.
authors.forename: Espen
authors.initials: E
authors.lastname: Melum
authors.affiliation: Division of Gastroenterology and Hepatology, Department of Medicine, University of Cambridge, Addenbrooek's Hospital, UK.
authors.forename: Arthur
authors.initials: A
authors.lastname: Kaser
authors.affiliation: 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.
authors.forename: Bertus
authors.initials: B
authors.lastname: Eksteen
authors.affiliation: Section of Gastroenterology, University of Milan-Bicocca, Milan, Italy; Liver Center, Yale University School of Medicine, United States.
authors.forename: Mario
authors.initials: M
authors.lastname: Strazzabosco
authors.affiliation: Division of Gastroenterology, Hepatology and Endocrinology, Medical University Hannover, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Manns
authors.affiliation: Hans Popper Laboratory of Molecular Hepatology, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Austria. Electronic address: michael.trauner@meduniwien.ac.at.
authors.forename: Michael
authors.initials: M
authors.lastname: Trauner
mesh_terms: D000818:Animals; D015209:Cholangitis, Sclerosing / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D004195:Disease Models, Animal; D006801:Humans
keywords: Animal model; Bile acids; Biliary fibrosis; Cholangiopathies; Cholestatic liver disease; Primary sclerosing cholangitis