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- W2165214298 abstract "Humans expressing a defective form of the transcription factor AIRE (autoimmune regulator) develop multiorgan autoimmune disease. We used aire- deficient mice to test the hypothesis that this transcription factor regulates autoimmunity by promoting the ectopic expression of peripheral tissue- restricted antigens in medullary epithelial cells of the thymus. This hypothesis proved correct. The mutant animals exhibited a defined profile of autoimmune diseases that depended on the absence of aire in stromal cells of the thymus. Aire-deficient thymic medullary epithelial cells showed a specific reduction in ectopic transcription of genes encoding peripheral antigens. These findings highlight the importance of thymically imposed central tolerance in controlling autoimmunity." @default.
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- W2165214298 date "2002-11-15" @default.
- W2165214298 modified "2023-10-15" @default.
- W2165214298 title "Projection of an Immunological Self Shadow Within the Thymus by the Aire Protein" @default.
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- W2165214298 doi "https://doi.org/10.1126/science.1075958" @default.
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