Transgenic mouse approaches to the pathogenesis of hereditary type of human autoimmune disease

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We sought to monitor the production and maintenance of Aire-expressing medullary thymic epithelial cells(mTECs) by a fate-mapping strategy in which bacterial artificial chromosome transgenic mice expressing Cre recombinase under the control of the Aire regulatory element were crossed with a GFP reporter strain. We found that, in addition to its well recognized expression within mature mTECs, Aire was expressed in the early embryo before emergence of the three germ cell layers. With the use of one transgenic line in which Cre recombinase expression was confined to mTECs, we found that Aire^+ CD80^<high> mTECs further progressed to an Aire^-CD80^<intermediate> stage, suggesting that Aire expression is not constitutive from after its induction until cell death but instead is down-regulated at the beginning of terminal differentiation.