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

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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.