Pathological features of new animal models for primary biliary cirrhosis.
Pathological features of new animal models for primary biliary cirrhosis.
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DOI：
10.1155/2012/403954
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发表时间：
2012
影响因子：
1.8
通讯作者：
Nakanuma Y
中科院分区：
文献类型：
--
作者：
Tsuneyama K;Moritoki Y;Kikuchi K;Nakanuma Y
原发性胆汁性肝硬化（PBC）是一种自身免疫性肝病，其特征是免疫介导的胆汁损伤和经常出现针对线粒体酶的自身抗体。目前几乎没有公认的、常规使用的动物模型，但近5年来，有几种独特的动物模型显示了人类PBC的特征性临床和病理特征。在此，我们比较了以前报道的和新引进的PBC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和理解导致了有希望的治疗方法和新工具的开发，以表征这些临床病症。此外，该模式是否适合预期目的，还需进一步研究和分析加以确认。
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.