Genetic control of the inflammatory T-cell response in regulatory T-cell deficient scurfy mice.
IPEX (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.
Sharma
Rahul
R
Department of Medicine, University of Virginia, Charlottesville, VA 22908, USA.
Ju
Shyr-Te
ST
D000818:Animals; D051858:Forkhead Transcription Factors / Q000235:genetics* / Q000378:metabolism; D005786:Gene Expression Regulation; D007249:Inflammation / Q000235:genetics* / Q000276:immunology*; D051379:Mice; D018345:Mice, Knockout; D008822:Mice, Transgenic; D009154:Mutation; D013601:T-Lymphocytes / Q000502:physiology*
D051858:Forkhead Transcription Factors; C498833:Foxp3 protein, mouse
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