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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.