Murine models of primary biliary cirrhosis: Comparisons and contrasts

The absence of suitable mouse models has represented a major drawback in our understanding of early events leading to the development of primary biliary cirrhosis (PBC). The recent report of not one, but three mouse models, each with distinctive features resembling PBC, represents a major advance in PBC research and generates novel experimental perspectives. The dnTGFbetaRII mouse, the IL-2Ralpha(-/-) mouse and the NOD.c3c4 mouse are all characterized by different genetic backgrounds and modifications, nonetheless all develop characteristic lymphocytic biliary infiltrates and specific antimitochondrial antibody response. Each model reflects characteristics of PBC in association with unique phenotypic differences. These three distinct models will allow a better understanding of the specific genetics involved in breaking of tolerance and progression of disease, in association with the possible definition of novel therapeutic approaches.