AIRE (F5F8X) Rabbit mAb #85045

Autoimmune disease is a significant health concern resulting from a breakdown in normal immune self-tolerance. Immune self-tolerance is regulated by a complex system that includes key cell types, regulatory molecules, cytokines, and intricate immune cell selection processes (1-3). The autoimmune regulator (AIRE) is a transcription factor first discovered in autoimmune disease patients with rare genetic mutations (4-6). AIRE has four distinct domains: a caspase recruitment domain (CARD), the SAND domain, and plant homeodomains 1 and 2 (PHD1 and PHD2) (2,3). AIRE plays a vital role in promoting self-tolerance in the thymus by regulating expression of common tissue-restricted peripheral self-antigens called tissue-restricted antigens (TSAs) (3). AIRE is primarily expressed by medullary thymic epithelial cells (mTECs), and coordinates expression of tissue-restricted proteins that are presented on major histocompatibility complex class I and II (MHC-I, MHC-II) and to developing T cells in the thymus (2,3). AIRE facilitates self-tolerance in other ways, such as regulating mTEC differentiation, controlling thymic dendritic cell accumulation, and developing regulatory T cells via XCL1 expression (3). AIRE regulates the production of CCR4 and CCR7 binding cytokines in mTECs and can alter thymocyte development and migration (2,3). In thymic B cells, AIRE is involved in self-antigen presentation for negative selection (2,3).