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pubmed-article:17046818pubmed:dateCreated2006-12-12lld:pubmed
pubmed-article:17046818pubmed:abstractTextMedullary thymic epithelial cells function as antigen-presenting cells in negative selection of self-reactive T cell clones, a process essential for the establishment of central self-tolerance. These cells mirror peripheral tissues through promiscuous expression of a diverse set of tissue-restricted self-antigens. The genes and signaling pathways that regulate the development of medullary thymic epithelial cells are not fully understood. Here we show that mice deficient in NF-kappaB2, a member of the NF-kappaB family, display a marked reduction in the number of mature medullary thymic epithelial cells that express CD80 and bind the lectin Ulex europaeus agglutinin-1, leading to a significant decrease in the extent of promiscuous gene expression in the thymus of NF-kappaB2(-/-) mice. Moreover, NF-kappaB2(-/-) mice manifest autoimmunity characterized by multiorgan infiltration of activated T cells and high levels of autoantibodies to multiple organs. A subpopulation of the mice also develops immune complex glomerulonephritis. These findings identify a physiological function of NF-kappaB2 in the development of medullary thymic epithelial cells and, thus, the control of self-tolerance induction.lld:pubmed
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pubmed-article:17046818pubmed:authorpubmed-author:PetersonPärtPlld:pubmed
pubmed-article:17046818pubmed:authorpubmed-author:DingJaneJlld:pubmed
pubmed-article:17046818pubmed:authorpubmed-author:WangZheZlld:pubmed
pubmed-article:17046818pubmed:authorpubmed-author:DingHan-FeiHFlld:pubmed
pubmed-article:17046818pubmed:authorpubmed-author:GunningWillia...lld:pubmed
pubmed-article:17046818pubmed:authorpubmed-author:ZhangBaochunBlld:pubmed
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pubmed-article:17046818pubmed:dateRevised2011-9-26lld:pubmed
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