Characterization of animal models for primary sclerosing cholangitis (PSC)

JOURNAL OF HEPATOLOGY

卷:60

Review

Fickert, Peter^1,2  Pollheimer, Marion J.^1,2  Beuers, Ulrich³  Lackner, Carolin²  Hirschfield, Gideon⁴  Housset, Chantal^5,6  Keitel, Verena⁷  Schramm, Christoph⁸  Marschall, Hanns-Ulrich⁹  Karlsen, Tom H.^10,11,12,13  Melum, Espen¹²  Kaser, Arthur¹⁴  Eksteen, Bertus^15,16  Strazzabosco, Mario^17,18  Manns, Michael¹⁹  Trauner, Michael²⁰ 

[1] Med Univ Graz, Dept Internal Med, Div Gastroenterol & Hepatol, Res Unit Expt & Mol Hepatol, Graz, Austria

[2] Med Univ Graz, Inst Pathol, Graz, Austria

[3] Univ Amsterdam, Acad Med Ctr, Tytgat Inst Liver & Intestinal Res, NL-1105 AZ Amsterdam, Netherlands

[4] Univ Birmingham, Sch Immun & Infect, Inst Biomed Res, Liver Res Ctr, Birmingham B15 2TT, W Midlands, England

[5] Univ Paris 06, F-75012 Paris, France

[6] INSERM, UMRS 938, Ctr Rech St Antoine, F-75012 Paris, France

[7] Med Univ Dusseldorf, Dept Internal Med, Div Gastroenterol & Hepatol, Dusseldorf, Germany

[8] Med Univ Hamburg Eppendorf, Hamburg, Germany

[9] Gothenburg Univ, Sahlgrenska Acad, Dept Mol & Clin Med, Wallenberg Lab, S-41124 Gothenburg, Sweden

[10] Natl Hosp Norway, Dept Med, Div Gastroenterol & Hepatol, Oslo, Norway

[11] Oslo Univ Hosp, Rikshosp, Norwegian PSC Res Ctr,Dept Transplantat Med, Div Canc Med Surg & Transplantat, Oslo, Norway

[12] Oslo Univ Hosp, Rikshosp, Internal Med Res Inst, Div Canc Med Surg & Transplantat, Oslo, Norway

[13] Univ Bergen, Dept Clin Med, Div Gastroenterol, Bergen, Norway

[14] Univ Cambridge, Addenbrookes Hosp, Dept Med, Div Gastroenterol & Hepatol, Cambridge CB2 2QQ, England

[15] Univ Birmingham, Sch Med, Inst Biomed Res, Ctr Liver Res,MRC,Ctr Immune Regulat, Birmingham, W Midlands, England

[16] Univ Hosp Birmingham NHS Trust, Queen Elizabeth Hosp, Birmingham, W Midlands, England

[17] Univ Milano Bicocca, Gastroenterol Sect, Milan, Italy

[18] Yale Univ, Sch Med, Ctr Liver, New Haven, CT 06510 USA

[19] Hannover Med Sch, Div Gastroenterol Hepatol & Endocrinol, Hannover, Germany

[20] Med Univ Vienna, Dept Internal Med 3, Div Gastroenterol & Hepatol, Hans Popper Lab Mol Hepatol, Vienna, Austria

关键词: Animal model;    Biliary fibrosis;    Bile acids;    Cholangiopathies;    Cholestatic liver disease;    Primary sclerosing cholangitis;   

DOI  :  10.1016/j.jhep.2014.02.006

来源: Elsevier

【 摘 要 】

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. (C) 2014 European Association for the Study of the Liver. Published by Elsevier B. V. All rights reserved.

【 授权许可】

Free