TY - JOUR
T1 - Animal Models of Autoimmune Liver Diseases
T2 - a Comprehensive Review
AU - Liu, Shou Pei
AU - Bian, Zhen Hua
AU - Zhao, Zhi Bin
AU - Wang, Jinjun
AU - Zhang, Weici
AU - Leung, Patrick S.C.
AU - Li, Liang
AU - Lian, Zhe Xiong
PY - 2020/1/1
Y1 - 2020/1/1
N2 - Autoimmune liver diseases (AILDs) are potentially life-threatening chronic liver diseases which include autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and recently characterized IgG4-related sclerosing cholangitis. They are caused by immune attack on hepatocytes or bile ducts, with different mechanisms and clinical manifestations. The etiologies of AILDs include a susceptible genetic background, environment insults, infections, and changes of commensal microbiota, but remain complicated. Understanding of the underlying mechanisms of AILDs is mandatory for early diagnosis and intervention, which is of great importance for better prognosis. Thus, animal models are developed to mimic the pathogenesis, find biomarkers for early diagnosis, and for therapeutic attempts of AILDs. However, no animal models can fully recapitulate features of certain AILD, especially the late stages of diseases. Certain limitations include different living condition, cell composition, and time frame of disease development and resolution. Moreover, there is no IgG4 in rodents which exists in human. Nevertheless, the understanding and therapy of AILDs have been greatly advanced by the development and mechanistic investigation of animal models. This review will provide a comprehensive overview of traditional and new animal models that recapitulate different features and etiologies of distinct AILDs.
AB - Autoimmune liver diseases (AILDs) are potentially life-threatening chronic liver diseases which include autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and recently characterized IgG4-related sclerosing cholangitis. They are caused by immune attack on hepatocytes or bile ducts, with different mechanisms and clinical manifestations. The etiologies of AILDs include a susceptible genetic background, environment insults, infections, and changes of commensal microbiota, but remain complicated. Understanding of the underlying mechanisms of AILDs is mandatory for early diagnosis and intervention, which is of great importance for better prognosis. Thus, animal models are developed to mimic the pathogenesis, find biomarkers for early diagnosis, and for therapeutic attempts of AILDs. However, no animal models can fully recapitulate features of certain AILD, especially the late stages of diseases. Certain limitations include different living condition, cell composition, and time frame of disease development and resolution. Moreover, there is no IgG4 in rodents which exists in human. Nevertheless, the understanding and therapy of AILDs have been greatly advanced by the development and mechanistic investigation of animal models. This review will provide a comprehensive overview of traditional and new animal models that recapitulate different features and etiologies of distinct AILDs.
KW - Animal models
KW - Autoimmune hepatitis
KW - Autoimmune liver disease
KW - IgG4-related sclerosing cholangitis
KW - Primary biliary cholangitis
KW - Primary sclerosing cholangitis
UR - http://www.scopus.com/inward/record.url?scp=85079760981&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85079760981&partnerID=8YFLogxK
U2 - 10.1007/s12016-020-08778-6
DO - 10.1007/s12016-020-08778-6
M3 - Review article
C2 - 32076943
AN - SCOPUS:85079760981
JO - Clinical Reviews in Allergy and Immunology
JF - Clinical Reviews in Allergy and Immunology
SN - 1080-0549
ER -