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. 2002 Nov 15;298(5597):1395-401.
doi: 10.1126/science.1075958. Epub 2002 Oct 10.

Projection of an immunological self shadow within the thymus by the aire protein

Mark S Anderson  1 , Emily S Venanzi, Ludger Klein, Zhibin Chen, Stuart P Berzins, Shannon J Turley, Harald von Boehmer, Roderick Bronson, Andrée Dierich, Christophe Benoist, Diane Mathis
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Projection of an immunological self shadow within the thymus by the aire protein

Mark S Anderson et al. Science. .

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.

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Comment in

  • Immunology. Exposing thy self.
    Ohashi PS. Ohashi PS. Science. 2002 Nov 15;298(5597):1348-9. doi: 10.1126/science.1079323. Science. 2002. PMID: 12434042 No abstract available.

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