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- W1624822079 endingPage "1983" @default.
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- W1624822079 abstract "T cell development is tightly controlled by thymic stromal cells. Alterations in stromal architecture affect T cell maturation and the development of self-tolerance. The monogenic autoimmune syndrome APECED (autoimmune-polyendocrinopathy-candidiasis-ectodermal dystrophy) is characterized by the loss of self-tolerance to multiple organs. Although mutations in the autoimmune regulator (AIRE) gene are responsible for this disease, the function of AIRE is not known. Here we report on the spatial and temporal pattern of murine Aire expression during thymic ontogeny and T cell selection. Early during development, thymic Aire transcription is critically dependent on RelB and occurs in epithelial cells in response to lymphocyte-mediated signals. In adult tissue, Aire expression is confined to the medulla and the corticomedullary junction, where it is modulated by thymocytes undergoing negative selection. Aire may determine thymic stromal organization and with it the induction of self-tolerance." @default.
- W1624822079 created "2016-06-24" @default.
- W1624822079 creator A5000393736 @default.
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- W1624822079 creator A5084319746 @default.
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- W1624822079 date "2000-08-15" @default.
- W1624822079 modified "2023-10-14" @default.
- W1624822079 title "Normal Thymic Architecture and Negative Selection Are Associated with <i>Aire</i> Expression, the Gene Defective in the Autoimmune-Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED)" @default.
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- W1624822079 doi "https://doi.org/10.4049/jimmunol.165.4.1976" @default.
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