Xu Liu
Yujia Zhang
Jingyao Huang
Dan Li
Bin Li
Shanghai Institute of Immunology, Department of Immunology and Microbiology, Shanghai Jiao Tong University School of Medicine, Shanghai Jiao Tong University, Shanghai, 200025, China
More InformationCorresponding author: E-mail address: danli@shsmu.edu.cn (Dan Li);E-mail address: binli@sibs.ac.cn (Bin Li)
Publish Date:2020-01-25
Abstract
Abstract
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-winged-helix 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.Keywords: IPEX syndrome,
Regulatory T cell,
Immune cell therapy,
FOXP3,
Post-translational modification,
Transcriptional complex ensemble
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