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Murine models of autoimmune cholangitis

机译：自身免疫性胆管炎的小鼠模型

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Purpose of review Primary biliary cirrhosis (PBC) is a human autoimmune liver disease whose molecular pathogenesis is poorly understood because of the difficulty in accessing human tissue and the absence of appropriate animal models. Recently, several unique murine models of human PBC have been discovered. These models have great potential for illustrating the cause and the cellular events that lead to biliary-specific damage. The purpose of this review is to summarize recent progress in these models. Recent findings The murine models of autoimmune cholangitis include the transforming growth factor beta receptor II (TGF-betaRII) dominant-negative (dnTGF-betaRII), IL-2 receptor a deleted (IL-2Ralpha~(-/-)), scurfy, nonobese diabetic (NOD) c3c4, and Ae2 gene-disrupted (Ae2_(a,b)~(-/-)) mice. Recently, we have also established a successful murine model following the immunization with a chemical mimicry of the lipoyl-lysine residue of the E2 component of PDC-E2. Summary These emerging murine models have greatly enabled researchers to address the pathogenesis of human PBC and to elucidate pathogenic factors. These models will ultimately lead to new therapeutic options for human PBC.
机译：审查目的原发性胆汁性肝硬化（PBC）是一种人类自身免疫性肝病，由于难以进入人体组织且缺乏合适的动物模型，因此对其分子发病机理的了解甚少。最近，已经发现了人类PBC的几种独特的鼠模型。这些模型具有很大的潜力来说明导致胆汁特异性损伤的原因和细胞事件。本文的目的是总结这些模型的最新进展。最近的发现自身免疫性胆管炎的小鼠模型包括转化生长因子β受体II（TGF-betaRII）显性阴性（dnTGF-betaRII），IL-2受体a缺失（IL-2Ralpha〜（-/-）），坏脾气非肥胖糖尿病（NOD）c3c4和Ae2基因中断（Ae2_（a，b）〜（-/-））小鼠。最近，在化学模拟PDC-E2的E2组分的脂酰赖氨酸残基后，我们还建立了成功的小鼠模型。总结这些新兴的小鼠模型极大地使研究人员能够解决人PBC的发病机理并阐明致病因素。这些模型最终将为人类PBC带来新的治疗选择。

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