Primary biliary cirrhosis and estrogen-mediated regulation of CD4+CD25+ thymocyte differentiation.

原发性胆汁性肝硬化是一种自身免疫性肝病合并慢性非化脓性破坏性胆管炎（CNSDC）。中年女性对这种疾病最敏感。在雌激素缺乏小鼠胸腺中，CD3+TCRbeta高胸腺细胞和CD4+CD25+胸腺细胞数量减少，CD4+CD8+CD25+胸腺细胞数量增加。雌激素缺乏小鼠的这些特征性病理特征导致老年小鼠自身反应性T细胞明显增加，可能是由于FOX3+ T细胞成熟过程受损。外源性雌激素完全消除了这些特征变化。雌激素缺乏小鼠胸腺器官培养是研究雌激素在体外胸腺细胞分化和成熟中的作用的有效方法。

Primary biliary cirrhosis is an autoimmune liver disease with chronic nonsupprative destructive cholangitis (CNSDC). Middle aged-female is most sensitive to this disease. In thymus of estrogen deficient mice, CD3+TCRbeta high thymocytes and CD4+CD25+thymocytes are decreased in number though CD4+CD8+CD25+thymocytes are increased in number. These characteristic pathological features in estrogen deficient mice results in an evident increase of autoreactive T cells in aged mice possibly due to impaired maturation process of FOX3+ T cell. Exogeneous administration of estrogen abrogates these characteristic changes completely. Organ culture of thymus derived from estrogen deficient mice is a potent method to investigate the role of estrogen in the differentiation and maturation of thymocytes in vitro.