Biallelic LOF mutations in AIRE, which are autosomally inherited, cause a defect in central tolerance with failure to delete auto-reactive lymphocytes in the thymus, manifesting in a syndrome characterized by chronic mucocutaneous candidiasis and multi-organ autoimmunity, particularly against parathyroid and adrenal glands (autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy, APECED, also called APS1) (63). This evidence concerns the gene AIRE and autoimmune polyendocrine syndrome type 1.