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自身免疫调节因子AIRE(autoimmune regulator,AIRE)是一种转录因子。Aire发生变异可导致人类出现APECED(autoimmune polyendocrinopathy candidiasis ectodermal dystrophy)疾病,患者表现为多种内分泌器官的特异性自身免疫性破坏,另外还有慢性皮肤黏膜念珠菌病和外胚层退行性病变等。AIRE-/-小鼠具有与APECED患者相似的自身免疫缺陷。2002年,Mark Anderson教授第一次明确了AIRE可以调节多种组织特异性抗原(TSA)在胸腺髓质上皮细胞(mTECs)的转录,从而启动阴性选择,维持T细胞的中枢耐受,解释了单一基因的突变却导致了多器官的免疫性损伤。AIRE是怎样特异
Autoimmune regulator AIRE (AIRE) is a transcription factor. Aire mutations can lead to the emergence of human APECED (autoimmune polyendocrinopathy candidiasis ectodermal dystrophy) disease, the patient showed a variety of endocrine organs specific autoimmune damage, in addition to chronic mucocutaneous candidiasis and ectodermal degeneration and so on. AIRE - / - mice have similar autoimmune deficits as APECED patients. In 2002, for the first time, Professor Mark Anderson made clear that AIRE can regulate the transcription of multiple tissue-specific antigens (TSA) in thymus medullary epithelial cells (mTECs), thereby initiating negative selection and maintaining central tolerance of T cells. Mutations in a single gene result in multiple organ damage. How AIRE is specific