原发性胆汁性胆管炎小鼠模型的特点及研究进展
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作者:
作者单位:

1.空军军医大学实验动物中心;2.空军军医大学西京医院消化内科

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中图分类号:

Q95

基金项目:

陕西省重点研发计划,国家自然科学基金项目(面上项目)


Characteristics and Research Advances in Mouse Models of Primary Biliary Cholangitis
Author:
Affiliation:

1.Laboratory Animal Center, Air Force Medical University;2.Department of Gastroenterology,Xijing Hospital,Air Force Medical University

Fund Project:

Key Research and Development Program of Shaanxi Province,The National Natural Science Foundation of China (General Program)

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    摘要:

    原发性胆汁性胆管炎(Primary biliary cholangitis, PBC)是一种高发于中年女性的自身免疫性疾病,其特征是免疫介导的肝内汇管区淋巴细胞浸润和胆管破坏。PBC的发病机制复杂,病因尚不明确。动物模型对于阐明PBC的发病机制及临床治疗具有重要意义。现有的PBC动物模型虽表现出与人类疾病发展类似的特征,但并未完全模拟PBC的临床表现和病理变化。本文系统比较了不同诱导方式构建的PBC小鼠模型在遗传背景、造模原理、致病细胞以及免疫学等方面的特征;比较了不同PBC小鼠模型免疫调控与疾病表型的差异;初步探讨了基于PBC动物模型的临床前治疗研究策略,有助于理解和优化动物模型,以进一步阐明人类PBC的复杂病理过程,为PBC的精准治疗提供新的思路。

    Abstract:

    Primary biliary cholangitis (PBC) is a prevalent autoimmune disease in middle-aged women, characterized by immune-mediated lymphocyte infiltration and bile duct destruction in intrahepatic bile ducts. The pathogenesis of PBC is complex, and the etiology is not yet clear. Animal models are meaningful for elucidating the pathogenesis and clinical treatment of PBC. Animal models cannot fully simulate the clinical manifestations and pathological changes of PBC, even the models show similar features of disease progression with human. Here, we systematically compare the heterogeneity of PBC mouse models constructed by different induction methods in terms of genetic background, modeling principles, pathogenic cells, and immune characteristics. Then, we review the differences in immune regulation and disease phenotypes among different PBC mouse models and preliminarily explore preclinical treatment strategies based on PBC mouse models. This work contributes to understanding and optimizing PBC mouse models, further elucidating the complex pathological processes of PBC, and providing new insights for the precise therapeutic intervention of PBC.

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  • 收稿日期:2025-03-14
  • 最后修改日期:2025-08-13
  • 录用日期:2025-10-17
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