Defects in the AIRE gene cause a monogenic autoimmune syndrome APECED
(autoimmune polyendocrinopathy candidiasis ectodermal dystrophy), which is characterized
by loss of self-tolerance to multiple organs. In concordance with its role in immune tolerance,
AIRE is most strongly expressed in thymic epithelial cells and in cells of monocytic-dendritic
lineage. The AIRE protein has been shown to function as a transcriptional regulator,
however, the mechanisms regulating AIRE gene expression are not known. Here we have …