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Trends Immunol. 2007 Jul;28(7):321-7. Epub 2007 Jun 7.

What's new in the Aire?

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  • 1Diabetes Center, University of California San Francisco, San Francisco, CA 94143, USA.

Abstract

Unraveling the mechanisms underlying autoimmune disease remains a difficult challenge. Recent lessons learned from the study of AIRE (autoimmune regulator), the gene responsible for the rare monogenic human syndrome APS-1, highlight the power of genetics to reveal disease pathogenesis. With the discovery of AIRE, central tolerance has re-emerged as a crucial check against autoimmunity. Aire-mediated regulation of diverse self-antigens in the thymus serves as a paradigm for the importance of promiscuous gene expression in the prevention of autoimmune disease. Recent characterization of Aire-targeted antigens continues to bear this out. Here, we review the current progress surrounding the role of Aire in central tolerance from a molecular, genetic and developmental basis.

PMID:
17556019
[PubMed - indexed for MEDLINE]
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