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Scurfy

gene symbol Sf. Scurfy mice display the most severe form of multi-organ inflammation due to a total lack of the CD4(+) FOXP3(+) regulatory T-cells (Tregs). The defect is caused by a mutation of the X-linked transcription factor FOXP3 (Wildin et al, 2001). X-linked neonatal diabetes mellitus, enteropathy, and endocrinopathy syndrome (IPEX), characterized also by fatal multi-organ inflammation, is the human equivalent of mouse scurfy (Bennett et al, 2001). In both instances the lack of this transcription factor leads to a complete absence of regulatory T-cells.

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