AIRE and autoimmune polyendocrine syndrome type 1: The first available mouse models of APECED syndrome were generated from various mouse strains and genetic backgrounds (summarized in Table 1 and Fig. 2) using two main approaches: 1) engineering genetic mutations found in human APECED patients into the murine Aire locus and, 2) using exon targeting to delete exons that encode functional domains of Aire (Fig. 2B).