title: Genetic control of the inflammatory T-cell response in regulatory T-cell deficient scurfy mice.
abstract: 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.
authors.affiliation: Department of Medicine, University of Virginia, Charlottesville, VA 22908, USA.
authors.forename: Rahul
authors.initials: R
authors.lastname: Sharma
authors.forename: Shyr-Te
authors.initials: ST
authors.lastname: Ju
mesh_terms: 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*
chemical_list: D051858:Forkhead Transcription Factors; C498833:Foxp3 protein, mouse
references.citation: J Immunol. 2007 Dec 15;179(12):8035-41
references.pmid: 18056343
references.citation: Nat Immunol. 2003 Apr;4(4):337-42
references.pmid: 12612581
references.citation: Annu Rev Immunol. 2008;26:205-32
references.pmid: 18304002
references.citation: Eur J Immunol. 1995 Jun;25(6):1617-23
references.pmid: 7614990
references.citation: Blood. 2007 Jan 1;109(1):383-5
references.pmid: 16990602
references.citation: Arthritis Rheum. 2007 Feb;56(2):509-20
references.pmid: 17265486
references.citation: Nat Genet. 2001 Jan;27(1):68-73
references.pmid: 11138001
references.citation: J Exp Med. 1995 Apr 1;181(4):1587-91
references.pmid: 7699339
references.citation: J Exp Med. 2004 May 3;199(9):1265-75
references.pmid: 15123746
references.citation: Proc Natl Acad Sci U S A. 2005 Oct 11;102(41):14735-40
references.pmid: 16203996
references.citation: J Exp Med. 2005 Sep 19;202(6):793-804
references.pmid: 16172258
references.citation: J Autoimmun. 2007 Aug;29(1):10-9
references.pmid: 17521882
references.citation: J Immunol. 1994 Oct 15;153(8):3764-74
references.pmid: 7930593
references.citation: Clin Exp Immunol. 2003 Aug;133(2):193-9
references.pmid: 12869024
references.citation: J Immunol. 2008 Sep 1;181(5):3285-90
references.pmid: 18714000
references.citation: Eur J Immunol. 2001 Jan;31(1):196-204
references.pmid: 11265635
references.citation: J Immunol. 2009 Jul 15;183(2):1065-73
references.pmid: 19553521
references.citation: J Immunol. 2008 Nov 15;181(10):6934-41
references.pmid: 18981113
references.citation: Curr Opin Immunol. 2005 Dec;17(6):638-42
references.pmid: 16209918
references.citation: J Exp Med. 2005 Nov 21;202(10):1387-97
references.pmid: 16301745
references.citation: Cell. 1996 Nov 29;87(5):811-22
references.pmid: 8945509
references.citation: Proc Natl Acad Sci U S A. 2004 Mar 16;101(11):3880-5
references.pmid: 15004274
references.citation: J Immunol. 2009 Jul 1;183(1):332-9
references.pmid: 19542444
references.citation: J Immunol. 2001 Dec 1;167(11):6217-24
references.pmid: 11714783
references.citation: J Immunol. 2009 Sep 1;183(5):3212-8
references.pmid: 19648271
references.citation: J Exp Med. 2006 Mar 20;203(3):777-88
references.pmid: 16533885
references.citation: Nat Immunol. 2003 Apr;4(4):330-6
references.pmid: 12612578
references.citation: Science. 1999 Nov 26;286(5445):1732-5
references.pmid: 10576739
references.citation: J Immunol. 2006 May 15;176(10):5839-47
references.pmid: 16670290
references.citation: J Med Genet. 2002 Aug;39(8):537-45
references.pmid: 12161590
references.citation: Int Immunol. 2006 Jul;18(7):991-1000
references.pmid: 16720616
references.citation: J Allergy Clin Immunol. 2005 Nov;116(5):1106-15
references.pmid: 16275384
references.citation: Immunol Rev. 2005 Apr;204:156-64
references.pmid: 15790357
references.citation: Int Immunol. 2008 Apr;20(4):517-23
references.pmid: 18310063
references.citation: Semin Immunol. 2004 Apr;16(2):81-8
references.pmid: 15036231
references.citation: Blood. 2007 Aug 15;110(4):1199-206
references.pmid: 17463170