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 th...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.展开更多
基金supported by National Natural Science Foundation of China (grants: 81830051,31525008, 31670911 and 31961133011)Shanghai Academic Research Leader 16XD1403800+1 种基金Shanghai Jiao Tong University (SJTU)-The Chinese University of Hong Kong (CUHK) Joint Research Collaboration Fundthe Fundamental Research Funds for Central Universities.
文摘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.
