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Hepatitis mouse models: from acute‐to‐chronic autoimmune hepatitis
Author(s) -
Yüksel Muhammed,
Laukens Debby,
Heindryckx Femke,
Van Vlierberghe Hans,
Geerts Anja,
Wong F. Susan,
Wen Li,
Colle Isabelle
Publication year - 2014
Publication title -
international journal of experimental pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.671
H-Index - 72
eISSN - 1365-2613
pISSN - 0959-9673
DOI - 10.1111/iep.12090
Subject(s) - autoimmune hepatitis , immunology , medicine , hepatitis , liver disease , immune system , autoantibody , fulminant , fulminant hepatitis , genetic predisposition , chronic liver disease , disease , liver injury , liver transplantation , transplantation , cirrhosis , pathology , antibody
Summary Autoimmune hepatitis ( AIH ) is a chronic inflammatory liver disease associated with interface hepatitis, raised plasma liver enzymes, the presence of autoantibodies and regulatory T‐cell (Tregs) dysfunction. The clinical course is heterogeneous, manifested by a fulminant or indolent course. Although genetic predisposition is well accepted, the combination with currently undefined environmental factors is crucial for the development of the disease. Progress in the development of reliable animal models provides added understanding of the pathophysiology of AIH , and these will be very useful in evaluating potential therapeutics. It appears that artificially breaking tolerance in the liver is easy. However, maintaining this state of tolerance breakdown, to get chronic hepatitis, is difficult because liver immune homeostasis is strongly regulated by several immune response inhibitory mechanisms. For example, Tregs are crucial regulators in acute and chronic hepatitis, and C57 BL /6 mice are most prone to experimental AIH . Immunization of C57 BL /6 mice with liver ( AIH ) autoantigens ( CYP 2D6/ FTCD or IL ‐4R) and the disturbance of liver regulatory mechanism(s), leading to experimental AIH , are likely to be most representative of human AIH pathology.