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AIRE expands: new roles in immune tolerance and beyond

2016; Nature Portfolio; Volume: 16; Issue: 4 Linguagem: Inglês

10.1038/nri.2016.9

1474-1741

Mark S. Anderson, Maureen A. Su,

T-cell and B-cell Immunology

Autoimmune regulator (AIRE) is best known for its role in immune tolerance. In this Review, the authors summarize the recent advances in our understanding of the diverse functions of AIRE, including its role in selection of regulatory T cells and modulation of non-autoimmune diseases. More than 15 years ago, mutations in the autoimmune regulator (AIRE) gene were identified as the cause of autoimmune polyglandular syndrome type 1 (APS1). It is now clear that this transcription factor has a crucial role in promoting self-tolerance in the thymus by regulating the expression of a wide array of self-antigens that have the commonality of being tissue-restricted in their expression pattern in the periphery. In this Review, we highlight many of the recent advances in our understanding of the complex biology that is related to AIRE, with a particular focus on advances in genetics, molecular interactions and the effect of AIRE on thymic selection of regulatory T cells. Furthermore, we highlight new areas of biology that are potentially affected by this key regulator of immune tolerance.