Abstract：
AIRE (for autoimmune regulator) is a multidomain protein that performs a fundamental function in the thymus and possibly in the secondary lymphoid organs: the regulation, especially in the sense of activation, of the process of gene transcription in cell lines deputed to the presentation of self-antigens to the maturing T lymphocytes. The apoptosis of the elements bearing T-cell receptors with critical affinity for the exhibited self-antigens prevents the escape of autoreactive clones and represents a simple and efficient mechanism of deletional self-tolerance. However, AIRE action relies on an articulated complex of biophysical and biochemical properties, in most cases attributable to single subspecialized domains. Here a thorough review of the matter is presented, with a privileged look at the pathogenic changes of AIRE that interfere with such properties and lead to the impairment in its chief function.
摘要：
AIRE（用于自身免疫调节剂）是一种多结构域蛋白，在胸腺中以及可能在次级淋巴器官中发挥基本功能：调节，特别是在激活的意义上，细胞系中的基因转录过程与自身抗原呈递到成熟的T淋巴细胞有关。带有T细胞受体的元件的凋亡对所显示的自身抗原具有关键亲和力，可防止自身反应性克隆的逃逸，并代表了一种简单有效的缺失自我耐受机制。然而,AIRE的作用依赖于生物物理和生化特性的复杂表达，在大多数情况下，可归因于单个亚专门领域。这里对此事进行了彻底的审查，特权观察AIRE的致病变化，这些变化会干扰这些特性并导致其主要功能受损。
