Transcriptional regulation by AIRE: molecular mechanisms of central tolerance

被引:176
作者
Peterson, Paert [1 ]
Org, Tonis [1 ]
Rebane, Ana [1 ]
机构
[1] Univ Tartu, Inst Gen & Mol Pathol, EE-50411 Tartu, Estonia
基金
英国惠康基金;
关键词
D O I
10.1038/nri2450
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
The negative selection of T cells in the thymus is necessary for the maintenance of self tolerance. Medullary thymic epithelial cells have a key function in this process as they express a large number of tissue-specific self antigens that are presented to developing T cells. Mutations in the autoimmune regulator (AIRE) protein cause a breakdown of central tolerance that is associated with decreased expression of self antigens in the thymus. In this Review, we discuss the role of AIRE in the thymus and recent advances in our understanding of how AIRE might function at the molecular level to regulate gene expression.
引用
收藏
页码:948 / 957
页数:10
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