Peterson Pärt, Org Tõnis, Rebane Ana
Institute of General and Molecular Pathology, University of Tartu, Tartu 5O411, Estonia.
Nat Rev Immunol. 2008 Dec;8(12):948-57. doi: 10.1038/nri2450.
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.
胸腺中T细胞的阴性选择对于维持自身耐受性至关重要。胸腺髓质上皮细胞在此过程中具有关键作用，因为它们表达大量组织特异性自身抗原，并将其呈递给发育中的T细胞。自身免疫调节因子（AIRE）蛋白的突变会导致中枢耐受性的破坏，这与胸腺中自身抗原表达的减少有关。在本综述中，我们讨论了AIRE在胸腺中的作用，以及我们对AIRE如何在分子水平上发挥作用以调节基因表达的最新认识进展。
