Animal models of primary biliary cirrhosis

Jinjun Wang, Guo Xiang Yang, Koichi Tsuneyama, M. Eric Gershwin, William M. Ridgway, Patrick S Leung

Research output: Contribution to journalArticlepeer-review

41 Scopus citations


Within the last decade, several mouse models that manifest characteristic features of primary biliary cirrhosis (PBC) with antimitochondrial antibodies (AMAs) and immune-mediated biliary duct pathology have been reported. Here, the authors discuss the current findings on two spontaneous (nonobese diabetic autoimmune biliary disease [NOD.ABD] and dominant negative transforming growth factor-β receptor II [dnTGFβRII]) and two induced (chemical xenobiotics and microbial immunization) models of PBC. These models exhibit the serological, immunological, and histopathological features of human PBC. From these animal models, it is evident that the etiology of PBC is multifactorial and requires both specific genetic predispositions and environmental insults (either xenobiotic chemicals or microbial), which lead to the breaking of tolerance and eventually liver pathology. Human PBC is likely orchestrated by multiple factors and hence no single model can fully mimic the immunopathophysiology of human PBC. Nevertheless, knowledge gained from these models has greatly advanced our understanding of the major immunological pathways as well as the etiology of PBC.

Original languageEnglish (US)
Pages (from-to)285-296
Number of pages12
JournalSeminars in Liver Disease
Issue number3
StatePublished - 2014


  • antimitochondrial antibodies
  • cholangiocytes
  • intrahepatic bile ducts
  • microbial immunization
  • xenobiotics

ASJC Scopus subject areas

  • Hepatology


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