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The parallel identification and characterization of patient cohorts with the monogenic disorder autoimmune polyendocrinopathy\u2010candidiasis\u2010ectodermal dystrophy (\n                    <jats:styled-content style=\"fixed-case\">APECED<\/jats:styled-content>\n                    ), which is typically caused by biallelic\n                    <jats:italic>\n                      <jats:styled-content style=\"fixed-case\">AIRE<\/jats:styled-content>\n                    <\/jats:italic>\n                    mutations, has underscored the critical contribution of\n                    <jats:styled-content style=\"fixed-case\">AIRE<\/jats:styled-content>\n                    in fungal immune surveillance at mucosal surfaces and in prevention of multiorgan autoimmunity in humans. In this review, we synthesize the current clinical, genetic, molecular and immunological knowledge derived from basic studies in Aire\u2010deficient animals and from\n                    <jats:styled-content style=\"fixed-case\">APECED<\/jats:styled-content>\n                    patient cohorts. We also outline major advances and research endeavors that show promise for informing improved diagnostic and therapeutic approaches for patients with\n                    <jats:styled-content style=\"fixed-case\">APECED<\/jats:styled-content>\n                    .\n                  <\/jats:p>","DOI":"10.1111\/imr.12714","type":"journal-article","created":{"date-parts":[[2018,12,18]],"date-time":"2018-12-18T20:12:24Z","timestamp":1545163944000},"page":"103-120","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":147,"title":["Lessons from primary immunodeficiencies: Autoimmune regulator and autoimmune polyendocrinopathy\u2010candidiasis\u2010ectodermal dystrophy"],"prefix":"10.1111","volume":"287","author":[{"given":"Gregory M.","family":"Constantine","sequence":"first","affiliation":[{"name":"Fungal Pathogenesis Section Laboratory of Clinical Immunology and Microbiology National Institute of Allergy and Infectious Diseases 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