AIRE(Autoimmune regulator) is a transcriptional regulator that forms nuclear bodies and interacts with the transcriptional coactivator CREB binding protein. AIRE plays a crucial role in the immune system by regulating self-antigen expression in the thymus, which is essential for self-tolerance development and autoimmune disease prevention. AIRE is localized in medullary epithelial cells and monocyte-dendritic cells of the thymus, where AIRE orchestrates transcriptional control of genes necessary for proper thymic environment establishment. This localization ensures developing T cells are exposed to diverse self-antigens, allowing autoreactive T cell elimination during negative selection. Mutations in AIRE can lead to impaired thymic T cell selection, contributing to autoimmune conditions like autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED).