Tsuneyama Koichi, Moritoki Yuki, Kikuchi Kentaro, Nakanuma Yasuni
Department of Diagnostic Pathology, Graduate School of Medicine and Pharmaceutical Science, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan.
Int J Hepatol. 2012;2012:403954. doi: 10.1155/2012/403954. Epub 2011 Jul 6.
Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by immune mediated biliary damage and frequent appearance of autoantibodies against mitochondrial enzymes. There is almost no useful animal model that is globally recognized and routinely used, however, several unique animal models manifested the characteristic clinical and pathological features of human PBC within the last 5 years. Herein, we compare the pathological features of previously reported and newly introduced novel animal models of PBC. Knowledge and understanding of the strengths and the limitations of each animal model have led to the development of promising therapies and novel tools to characterize these clinical conditions. Moreover, suitability of the model for the intended purpose should be confirmed by further research and analysis.
原发性胆汁性肝硬化（PBC）是一种自身免疫性肝病，其特征为免疫介导的胆汁损伤以及针对线粒体酶的自身抗体频繁出现。然而，几乎没有全球公认且常规使用的有用动物模型，不过，在过去5年中，几种独特的动物模型表现出了人类PBC的特征性临床和病理特征。在此，我们比较先前报道的和新引入的PBC新型动物模型的病理特征。对每种动物模型的优势和局限性的了解促成了有前景的治疗方法和用于表征这些临床病症的新工具的开发。此外，该模型对于预期目的的适用性应通过进一步的研究和分析来确认。
