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Molecular feature and therapeutic perspectives of immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome

查看全文 作　　者：Qianru [1]Huang;Xu [1]Liu;Yujia [1]Zhang;Jingyao [1]Huang;Dan [1]Li;Bin [1]Li 高影响力作者 机构地区：[1]Shanghai Institute of Immunology,Department of Immunology and Microbiology,Shanghai Jiao Tong University School of Medicine,Shanghai Jiao Tong University,Shanghai,200025,China高影响力机构 出　　处：《Journal of Genetics and Genomics》索引2020年第47卷第1期,共10页高影响力期刊 基　　金：supported by National Natural Science Foundation of China (grants: 81830051,31525008, 31670911 and 31961133011);Shanghai Academic Research Leader 16XD1403800;Shanghai Jiao Tong University (SJTU)-The Chinese University of Hong Kong (CUHK) Joint Research Collaboration Fund;the Fundamental Research Funds for Central Universities. 摘　　要：Regulatory T(Treg) cells, a subtype of immunosuppressive CD4^+T cells, are vital for maintaining immune homeostasis in healthy people. Forkhead box protein P3(FOXP3), a member of the forkhead-wingedhelix family, is the pivotal transcriptional factor of Treg cells. The expression, post-translational modifications, and protein complex of FOXP3 present a great impact on the functional stability and immune plasticity of Treg cells in vivo. In particular, the mutation of FOXP3 can result in immune dysregulation,polyendocrinopathy, enteropathy, X-linked(IPEX) syndrome, which is a rare genetic disease mostly diagnosed in early childhood and can soon be fatal. IPEX syndrome is related to several manifestations,including dermatitis, enteropathy, type 1 diabetes, thyroiditis, and so on. Here, we summarize some recent findings on FOXP3 regulation and Treg cell function. We also review the current knowledge about the underlying mechanism of FOXP3 mutant-induced IPEX syndrome and some latest clinical prospects.At last, this review offers a novel insight into the role played by the FOXP3 complex in potential therapeutic applications in IPEX syndrome. 关 键 词：IPEX SYNDROME Regulatory T CELL Immune CELL therapy FOXP3 POST-TRANSLATIONAL modification Transcriptional complex ensemble
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