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Nature Reviews Immunology 7, 645–650 (1 August 2007) | doi:10.1038/nri2136

A decade of AIRE

Diane Mathis & Christophe Benoist

In 1997, the autoimmune regulator (AIRE) gene was identified as the locus underlying susceptibility to the polyendocrine autoimmune disease known as autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). In the intervening 10 years, it has become increasingly clear that this rare disorder has provided us with an illuminative window on one of the most fundamental processes of the immune system — the establishment and maintenance of self tolerance.