小鼠原发性胆汁性胆管炎模型及其在疾病研究中的作用

Murine models of primary biliary cholangitis and its role in disease research

原发性胆汁性胆管炎(primary biliary cholangitis,PBC)是一种多发于女性的肝脏自身免疫性疾病,其特征是免疫介导肝内小胆管的破坏和抗线粒体抗体(AMA)的存在。由于获取人体组织标本的困难和缺乏合适的动物模型,目前,在了解该病的发病机制方面进展有限。近些年来,一些独特的小鼠模型被先后报道,这些小鼠模型显示出类似于人类PBC的血清学、生化和组织学特征。该文对各种PBC的小鼠模型进行综述,详细介绍各种小鼠PBC模型特点和形成机制,并讨论它们在PBC研究中的优势和局限,为研究PBC的发病机制和开发PBC的治疗药物提供重要的理论依据。

Primary biliary cholangitis(PBC)is an autoimmune liver disease characterized by destructive cholangitis affecting the small intrahepatic bile ducts,and the presence of antimitochondrial antibodies(AMA).At present,there has been limited progress in exploring the pathogenesis of the disease,because of difficulty in obtaining human tissue samples and lack of suitable animal models.In recent years,some murine models have been reported,which showed serological,biochemical,and histological characteristics similar to those of humans with PBC.In this review,various murine models of PBC are reviewed,and the characteristics and mechanism of PBC in mice are introduced.Besides,their advantages and limitations in the study of PBC are discussed.It hopes to provide an important theoretical basis for studying the pathogenesis of PBC and developing therapeutic drugs for PBC.