直近一年間の累計
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ID 117351
著者
Xu, Ya-Fei South China University of Technology
Yao, Yuan South China University of Technology
Ma, Min South China University of Technology
Yang, Shu-Han South China University of Technology
Jiang, Peng Southeast University
Wang, Jinjun Yangzhou University
Wang, Chan Southeast University
Liu, Xiangdong Southeast University
Li, Liang Guangdong Academy of Medical Sciences
Lian, Zhe-Xiong South China University of Technology
資料タイプ
学術雑誌論文
抄録
Primary biliary cholangitis (PBC) is a cholestatic liver disease primarily featured by autoimmune-mediated damage of intrahepatic small- and medium-sized bile ducts. Elevated serum proinflammatory cytokines, serum anti-mitochondrial antibodies (AMAs), liver inflammation, and fibrosis are also hallmarks of PBC disease. However, whether the elevated proinflammatory cytokines play a role in autoimmune cholangitis remains unknown. Herein, we utilized the p40-/-IL-2Rα-/- PBC mouse model to investigate the roles of proinflammatory cytokines IL-18, IL-21, and IFN-γ in the onset and progression of PBC. IL-18-/-, IFN-γ-/-, and IL-21-/- mice were crossed with p40-/-IL-2Ra+/- mice, respectively, to produce corresponding cytokine-deficient PBC models. Autoantibody level, liver inflammation, and bile duct injury were analyzed. We found that livers from p40-/-IL-2Rα-/- mice exhibit similar transcriptomic characters of PBC patients. In p40-/-IL-2Rα-/- mice, deletion of IL-18 has no remarkable effect on disease progression, while deletion of IL-21 indicates that it is necessary for AMA production but independent of liver inflammation and cholangitis. IFN-γ is responsible for both AMA production and liver inflammation in our model. Our results demonstrate that different proinflammatory cytokines can regulate different effector functions in PBC pathogenesis and need to be considered in PBC treatment.
掲載誌名
Journal of Immunology Research
ISSN
23148861
23147156
出版者
Hindawi
2022
開始ページ
7111445
発行日
2022-03-07
権利情報
This is an open access article distributed under the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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フルテキストファイル
言語
eng
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出版社版
部局
医学系