biostudies-literatureWarren BDNICHD NIH HHSNational Institutes of HealthNIGMS NIH HHSIDeA Network of Biomedical Research Excellence1492-1504https://www.ebi.ac.uk/biostudies/studies/S-EPMC6561863biostudies-literatureUnknown100(6)Autoimmune Regulator (AIRE) regulates central immune tolerance by inducing expression of tissue-restricted antigens in thymic medullary epithelial cells, thereby ensuring elimination of autoreactive T cells. Aire mutations in humans and targeted Aire deletion in mice result in multiorgan autoimmune disease, known in humans as autoimmune polyglandular syndrome type 1 (APS-1). APS-1 is characterized by the presence of adrenal insufficiency, chronic mucosal candidiasis, and/or hypoparathyroidism. Additionally, females often present with gonadal insufficiency and infertility. Aire-deficiency (KO) in mice results in oophoritis and age-dependent depletion of follicular reserves. Here, we found that while the majority of young 6-week-old Aire-KO females had normal follicular reserves, mating behaBiology of reproductionAutoimmune Regulator is required in female mice for optimal embryonic development and implantation†.PMC6561863GM103418P20 GM104936P20 GM103418R21 HD082484HD042280R01 HD045611HD082484R21 HD062879HD045611R01 HD042280HD062879Grzesiak GSu RWPetroff BKPetroff MGMcGinnis LKAhn SHWarren BDFazleabas ATChristenson LKfalseAutoimmune Regulator is required in female mice for optimal embryonic development and implantation†.Autoimmune Regulator (AIRE) regulates central immune tolerance by inducing expression of tissue-restricted antigens in thymic medullary epithelial cells, thereby ensuring elimination of autoreactive T cells. Aire mutations in humans and targeted Aire deletion in mice result in multiorgan autoimmune disease, known in humans as autoimmune polyglandular syndrome type 1 (APS-1). APS-1 is characterized by the presence of adrenal insufficiency, chronic mucosal candidiasis, and/or hypoparathyroidism. Additionally, females often present with gonadal insufficiency and infertility. Aire-deficiency (KO) in mice results in oophoritis and age-dependent depletion of follicular reserves. Here, we found that while the majority of young 6-week-old Aire-KO females had normal follicular reserves, mating beha2019-01-01T00:00:00Z2019 Jun2025-04-18T22:08:58.323Z2025-04-07T09:58:16.073ZS-EPMC65618633077053210.1093/biolre/ioz023