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      Fibrosis in Autoimmune and Cholestatic Liver Disease

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          Abstract

          Autoimmune and cholestatic liver disease account for a significant part of end-stage liver disease and are leading indications for liver transplantation. Especially cholestatic liver diseases (primary biliary cirrhosis and primary sclerosing cholangitis) appear to be different from other chronic liver diseases with regards to pathogenesis. Portal fibroblasts located in the connective tissue surrounding bile ducts appear to be different from hepatic stellate cells with regards to expression of marker proteins and response the profibrogenic and mitogenic stimuli. In addition there is increasing evidence for a cross talk between activated cholangiocytes and portal myofibroblasts. Several animal models have improved our understanding of the mechanisms underlying these chronic liver diseases. In the present review, we discuss the current concepts and ideas with regards to myofibroblastic cell populations, mechanisms of fibrosis, summarize characteristic histological findings and currently employed animal models of autoimmune and cholestatic liver disease.

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          Liver fibrosis.

          Ramón Bataller, David A. Brenner (2005)
          Liver fibrosis is the excessive accumulation of extracellular matrix proteins including collagen that occurs in most types of chronic liver diseases. Advanced liver fibrosis results in cirrhosis, liver failure, and portal hypertension and often requires liver transplantation. Our knowledge of the cellular and molecular mechanisms of liver fibrosis has greatly advanced. Activated hepatic stellate cells, portal fibroblasts, and myofibroblasts of bone marrow origin have been identified as major collagen-producing cells in the injured liver. These cells are activated by fibrogenic cytokines such as TGF-beta1, angiotensin II, and leptin. Reversibility of advanced liver fibrosis in patients has been recently documented, which has stimulated researchers to develop antifibrotic drugs. Emerging antifibrotic therapies are aimed at inhibiting the accumulation of fibrogenic cells and/or preventing the deposition of extracellular matrix proteins. Although many therapeutic interventions are effective in experimental models of liver fibrosis, their efficacy and safety in humans is unknown. This review summarizes recent progress in the study of the pathogenesis and diagnosis of liver fibrosis and discusses current antifibrotic strategies.
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            Primary biliary cirrhosis.

            Marshall M Kaplan, M. Gershwin (2005)
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              Abrogation of TGFbeta signaling in T cells leads to spontaneous T cell differentiation and autoimmune disease.

              L Gorelik, R A Flavell (2000)
              Targeted mutation of TGFbeta1 in mice demonstrated that TGFbeta1 is one of the key negative regulators of immune homeostasis, as its absence leads to activation of a self-targeted immune response. Nevertheless, because of the highly pleiotropic properties of TGFbeta and the presence of TGFbeta receptors on most cell types, its biologic role in the regulation of immune homeostasis is not yet understood. To limit the consequences of TGFbeta effects to a single cell type, we developed a transgenic approach to abrogate the TGFbeta response in key immune cells. Specifically, we expressed a dominant-negative TGFbeta receptor type II under a T cell-specific promoter and created a mouse model where signaling by TGFbeta is blocked specifically in T cells. Using this transgenic model, we show that T cell homeostasis requires TGFbeta signaling in T cells.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Best Pract Res Clin Gastroenterol
                Journal ID (iso-abbrev): Best Pract Res Clin Gastroenterol
                Title: Best Practice & Research. Clinical Gastroenterology
                Publisher: Elsevier
                ISSN (Print): 1521-6918
                ISSN (Electronic): 1532-1916
                Publication date PMC-release: April 2011
                Publication date (Print): April 2011
                Volume: 25
                Issue: 2-4
                Pages: 245-258
                Affiliations
                [a ]Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria
                [b ]Institute of Pharmacology, Center for Physiology and Pharmacology, Medical University of Vienna, Austria
                Author notes
                [∗ ]Corresponding author. Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 43 18-20, A-1090 Vienna, Austria. Tel.: +43 140 400 4741; fax: +43 140 400 4735. Michael.Trauner@ 123456meduniwien.ac.at
                Article
                Publisher ID: YBEGA1007
                DOI: 10.1016/j.bpg.2011.02.001
                PMC ID: 3134112
                PubMed ID: 21497742
                SO-VID: a6c936d9-1b93-4088-ba3c-e60d2fce6233
                Copyright © © 2011 Elsevier Ltd.
                License:

                This document may be redistributed and reused, subject to certain conditions.

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                Subject: 5

                ScienceOpen disciplines: Gastroenterology & Hepatology
                Keywords: fibrosis,cirrhosis,primary sclerosing cholangitis,hepatic stellate cells,cholestasis,primary biliary cirrhosis,portal myofibroblasts
                Data availability:
                ScienceOpen disciplines: Gastroenterology & Hepatology
                Keywords: fibrosis, cirrhosis, primary sclerosing cholangitis, hepatic stellate cells, cholestasis, primary biliary cirrhosis, portal myofibroblasts

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