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lüll Genetic control of the inflammatory T-cell response in regulatory T-cell deficient scurfy mice Sharma R; Ju STClin Immunol 2010[Aug]; 136 (2): 162-9IPEX (Immunodysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome is a rare, recessive disorder in patients with mutations in the foxp3 gene, the normal expression of which is required for the generation of functional regulatory T-cells. Scurfy mice also bear a mutation in the foxp3, and like IPEX patients, spontaneously develop multi-organ inflammation. As reviewed herein, breeding immune response genes into Scurfy mice has provided useful insight into how the inflammatory T-cell response is regulated in the absence of regulatory T-cells and post regulatory T-cell checkpoint. Of particular interest are those that preferentially affect the inflammatory T-cell response in an "apparent" organ-specific manner, implying that specific mechanisms of control exist for individual organs during multi-organ inflammation.|Animals[MESH]|Forkhead Transcription Factors/*genetics/metabolism[MESH]|Gene Expression Regulation[MESH]|Inflammation/*genetics/*immunology[MESH]|Mice[MESH]|Mice, Knockout[MESH]|Mice, Transgenic[MESH]|Mutation[MESH]|T-Lymphocytes/*physiology[MESH] |