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Experimental evidence on the immunopathogenesis of primary biliary cirrhosis

Abstract

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease for which an autoimmune pathogenesis is supported by clinical and experimental data, including the presence of autoantibodies and autoreactive T cells. The etiology remains to be determined, yet data suggest that both a susceptible genetic background and unknown environmental factors determine disease onset. Multiple infectious and chemical candidates have been proposed to trigger the disease in a genetically susceptible host, mostly by molecular mimicry. Most recently, several murine models have been reported, including genetically determined models as well as models induced by immunization with xenobiotics and bacteria.

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Selmi, C., Meda, F., Kasangian, A. et al. Experimental evidence on the immunopathogenesis of primary biliary cirrhosis. Cell Mol Immunol 7, 1–10 (2010). https://doi.org/10.1038/cmi.2009.104

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