motifs/domains
| a N-terminal homogeneously staining region (HSR) of Sp100 and Sp140 proteins and functioning as a dimerization domain, this HSR/CARD domain together with a nuclear localization signal is sufficient to induce apoptosis  |
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a DEAF-1 domain |
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two nuclear localization signals (NLS) |
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two putative DNA binding sequences referred as the SAND domain |
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a proline-rich region between |
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two PHD-type (C4-H-C3) zinc finger domains (the two PHD domains can bind to specific DNA sequence motifs), and PHD2 was required for AIRE to interact with sets of protein partners involved in chromatin structure/binding or transcription but not with those implicated in pre-mRNA processing |
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a leucine zipper motif within the first PHD domain |
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four dispersed nuclear receptor-binding LXXLL motifs |
| basic FUNCTION
| may be involved in the regulation of gene expression |
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autoimmune regulator gene, transactivating transcription through heteromeric molecular interactions regulated by homomultimerization |
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AIRE regulates gene expression by recruiting components of the transcription complex to specific regions of the genome via interactions with nuclear matrix |
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promotes the establishment of self-tolerance by regulating gene expression in the thymus |
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with PIAS1 interact functionally to regulate the activities of the target genes of AIRE |
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functions as a transcriptional regulator, and has a central role in the development of immunological tolerance |
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has a critical role in dendritic cells responses to microbial stimuli |
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able to promote ectopic gene expression from chromatin associated with histone modifications characteristic to inactive genes, and of tissue-specific genes from chromatin lacking transcriptionally active histone H3 modifications |
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may be involved in structural changes of larger chromatin regions |
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regulates the expression of differentiation-associated genes and self-renewal of embryonic stem cells |
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transcription factor involved in the presentation of tissue-restricted antigens during T-cell development in the thymus |
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transcription factor that induces the expression of a large subset of otherwise strictly tissue restricted antigens in medullary thymic epithelial cells, thereby enabling their presentation to developing T cells for negative selection |
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AIRE requires CDK9 to activate transcription elongation and co-transcriptional processing of target genes |
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has a central role in the transcriptional regulation of self-antigens in medullary thymic epithelial cells, which is necessary for negative selection of autoreactive T cells |
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the pathways behind AIRE-induced apoptosis may be directly linked to the induction of central tolerance in thymus |
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AIRE-induced cellular stress and apoptosis are associated with GAPDH translocation into the nuclei |
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impacts immunological tolerance by regulating the expression of a large set of genes in thymic medullary epithelial cells |
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multidomain protein that performs a fundamental function in the thymus and possibly in the secondary lymphoid organs: the regulation, especially in the sense of activation, of the process of gene transcription in cell lines deputed to the presentation of self-antigens to the maturing T lymphocytes |
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plays a remarkable role as a regulator of central tolerance by controlling the promiscuous expression of tissue-specific antigens in thymic medullary epithelial cells |
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fundamental role of AIRE and promiscuous gene expression (pGE), namely, central tolerance, in the predisposition to autoimmunity of Down syndrome individuals |
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key factor in thymic negative selection of autoreactive T cells by promoting the ectopic expression of tissue-specific genes in the thymic medullary epithelium |
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presence of AIRE can trigger molecular events leading to an altered chromatin landscape and the enhanced transcription of low-expressed genes |
