原发性胆汁性胆管炎小鼠模型的研究进展

Research advances in the mouse model of primary biliary cholangitis

原发性胆汁性胆管炎(PBC)是一种以小叶间胆管破坏,炎性因子浸润为主要特征的自身免疫性疾病。PBC以抗线粒体抗体升高为主要特点,最终导致肝内胆管胆汁淤积、肝纤维化甚至肝硬化。该病目前发病机制尚未完全清楚,也无理想的治疗方案。因此,有必要寻找一种与PBC相匹配的动物模型对PBC的发病、治疗、转归进行研究。简述胆管结扎模型、药物模型、抗原模型、基因自发模型等方面的进展,旨在总结归纳近年来发表的PBC小鼠模型的特点与局限,为PBC模型的选择和研究提供依据。

Primary biliary cholangitis （PBC） is an autoimmune disease characterized by the destruction of interlobular bile ducts and infil- tration of inflammatory factors. An increase in anti - mitochondrial antibody is the major feature of PBC and may eventually lead to intrahepatic cholestasis, liver fibrosis, and even liver cirrhosis. The pathogenesis of this disease remains unclear, and there is no ideal treatment regimen. Therefore, it is necessary to find an animal model matching with PBC, in order to study the pathogenesis, treatment, and prognosis of PBC. This article introduces the research advances in bile duct ligation models, drug models, antigen models, gene models, and spontaneous models, in order to summarize the features and limitations of the mouse model of PBC in the articles published in recent years and provide a basis for the selection and research of PBC models.