Defects in the autoimmune regulator (AIRE) gene cause the monogenic autoimmune
disease autoimmune polyendocrinopathy syndrome type 1 (APS-1), which is characterized
by a loss of self-tolerance to multiple organs. In concordance with its role in immune
tolerance, AIRE is strongly expressed in medullary thymic epithelial cells (mTECs). Data on
mechanisms controlling AIRE activation and the expression of this gene in other tissues are
fragmentary and controversial. We report here AIRE mRNA expression profiling of a large …