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Primary biliary cirrhosis (PBC) is a chronic liver disease caused by progressively immune-mediated liver injury.PBC is characterized by disorder of immune balance,as increased infiltration of lymphocytes and up-regulated level of Th1 cytokines.The biliary disease often relapses in PBC patients even after liver transplantation.Effective therapeutic strategies for autoimmune liver disease are limited because of its promiscuous pathogenesis.We took advantage of a mouse model which will develop spontaneous autoimmune liver disease and progress into liver fibrosis and clarified the mechanism of immune disorder in autoimmune liver disease.