Functional analysis of the gene responsible for the hereditary type of human autoimmune disease

Aire是一种遗传性自身免疫性疾病的基因。阐明Aire缺陷型自身免疫性疾病的发病机制将有助于揭示自身免疫性疾病的发病机制。为了了解Aire是如何建立免疫耐受的，我们构建了实验系统，在该系统中我们获得了Aire表达细胞的细胞动力学信息。出乎意料的是，我们发现Aire在胸腺器官发生之前的早期胚胎中表达。此外，我们证明了表达Aire的细胞的半衰期约为1周，这比以前认为的要长得多。

Aire is a gene responsible for the hereditary type of human autoimmune disease. Elucidation of the mechanisms underlying the Aire-deficient autoimmune pathogenesis would contribute to unveiling the pathogenesis of autoimmune disease. In order to know how the Aire establishes the immune tolerance, we constructed the experimental system where we get the information on the cellular dynamics of Aire-expressing cells. Unexpectedly, we found that Aire is expressed by early embryos prior to the thymic organogenesis. Furthermore, we demonstrated that half-life of Aire-expressing cells is about 1 week, which turned out to be much longer than previously thought.