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 journalArticle

34 Citations (Scopus)

Abstract

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
Volume34
Issue number3
DOIs
StatePublished - 2014

Fingerprint

Biliary Liver Cirrhosis
Animal Models
Xenobiotics
Pathology
Growth Factor Receptors
Transforming Growth Factors
Genetic Predisposition to Disease
Autoimmune Diseases
Immunization
Antibodies
Liver

Keywords

  • antimitochondrial antibodies
  • cholangiocytes
  • dnTGFBRII
  • intrahepatic bile ducts
  • microbial immunization
  • NOD.ABD
  • xenobiotics

ASJC Scopus subject areas

  • Hepatology

Cite this

Animal models of primary biliary cirrhosis. / Wang, Jinjun; Yang, Guo Xiang; Tsuneyama, Koichi; Gershwin, M. Eric; Ridgway, William M.; Leung, Patrick S.

In: Seminars in Liver Disease, Vol. 34, No. 3, 2014, p. 285-296.

Research output: Contribution to journalArticle

Wang, Jinjun ; Yang, Guo Xiang ; Tsuneyama, Koichi ; Gershwin, M. Eric ; Ridgway, William M. ; Leung, Patrick S. / Animal models of primary biliary cirrhosis. In: Seminars in Liver Disease. 2014 ; Vol. 34, No. 3. pp. 285-296.
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