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Murine models of primary biliary cirrhosis: Comparisons and contrasts
Author(s) -
Oertelt Sabine,
Ridgway William M.,
Ansari Aftab A.,
Coppel Ross L.,
Gershwin M. Eric
Publication year - 2007
Publication title -
hepatology research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.123
H-Index - 75
eISSN - 1872-034X
pISSN - 1386-6346
DOI - 10.1111/j.1872-034x.2007.00226.x
Subject(s) - primary biliary cirrhosis , primary (astronomy) , medicine , biliary cirrhosis , gastroenterology , physics , disease , astronomy , autoimmune disease
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 dnTGFβRII mouse, the IL‐2Rα –/– 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.