منابع مشابه
Aire.
Mutations in the transcriptional regulator, Aire, cause APECED, a polyglandular autoimmune disease with monogenic transmission. Animal models of APECED have revealed that Aire plays an important role in T cell tolerance induction in the thymus, mainly by promoting ectopic expression of a large repertoire of transcripts encoding proteins normally restricted to differentiated organs residing in t...
متن کاملThe AIRE -230Y Polymorphism Affects AIRE Transcriptional Activity: Potential Influence on AIRE Function in the Thymus
BACKGROUND The autoimmune regulator (AIRE) is expressed in the thymus, particularly in thymic medullary epithelial cells (mTECs), and is required for the ectopic expression of a diverse range of peripheral tissue antigens by mTECs, facilitating their ability to perform negative selection of auto-reactive immature T-cells. The expression profile of peripheral tissue antigens is affected not only...
متن کاملTwenty Years of AIRE
About two decades ago, cloning of the autoimmune regulator (AIRE) gene materialized one of the most important actors on the scene of self-tolerance. Thymic transcription of genes encoding tissue-specific antigens (ts-ags) is activated by AIRE protein and embodies the essence of thymic self-representation. Pathogenic AIRE variants cause the autoimmune polyglandular syndrome type 1, which is a ra...
متن کاملEstrogen turns down "the AIRE".
Genetic alterations are known drivers of autoimmune disease; however, there is a much higher incidence of autoimmunity in women, implicating sex-specific factors in disease development. The autoimmune regulator (AIRE) gene contributes to the maintenance of central tolerance, and complete loss of AIRE function results in the development of autoimmune polyendocrinopathy syndrome type 1. In this i...
متن کاملAire, Master of Many Trades
In thymic epithelial cells, the protein Aire (autoimmune regulator) induces the ectopic expression of hundreds of peripheral tissue antigens, thus enlarging the repertoire of antigens available for the induction of central T cell tolerance. By analyzing Aire's interacting partners, Abramson et al. (2010) shed new light on this unorthodox form of gene expression.
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ژورنال
عنوان ژورنال: Journal of Experimental Medicine
سال: 2005
ISSN: 1540-9538,0022-1007
DOI: 10.1084/jem2026iti1