Aire-mediated mechanism of tolerance against Dsg3, the autoantigen in pemphigus vulgaris

转录因子Aire促进胸腺中广泛的外周器官特异性抗原的表达，这些抗原将在负选择中作为自身抗原。我们发现寻常型天疱疮自身抗原桥粒芯糖蛋白3(Dsg3)依赖于Aire在小鼠胸腺中表达。Aire^&lt；-/-&gt；T细胞，而不是Aire^&lt；+/+&gt；T细胞，当与Dsg3^&lt；-/-&gt；B细胞一起转移时，在Rag2^&lt；-/-&gt；小鼠体内诱导低水平的抗Dsg3抗体产生。这些发现表明，Aire通过选择胸腺中的T细胞在对Dsg3的耐受中发挥作用。

The transcription factor Aire promotes the expression of a wide range of peripheral organ-specific antigens in the thymus, which will serve as self-antigens in negative selection. We demonstrated desmoglein 3 (Dsg3), the autoantigen in pemphigus vulgaris, expressed in mouse thymus dependent on Aire. Aire^<-/-> T cells, but not Aire^<+/+> T cells, induced low levels of anti-Dsg3 IgG production when transferred with Dsg3^<-/-> B cells in Rag2^<-/-> recipient mice. These findings suggest that Aire plays a role in tolerance against Dsg3 through the selection of T cells in thymus.