The monogenetic autoimmune disease autoimmune polyendocrinopathy syndrome type 1 (APS1) (also called autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED)) is a multiple organ-specific autoimmune disease in which a mutation in the autoimmune regulator (AIRE) gene is causative to the breach of central immune tolerance [5]. The gene discussed is AIRE; the disease is autoimmune polyendocrine syndrome type 1.