JAX
Mouse Strain Datasheet - 006775
NOD.Cg-Foxp3sf/DoiJ
Also Known As: NOD-scurfy, NOD.Fox-p3
Hemizygous males carrying the recessive, spontaneous scurfy mutation exhibit massive lymphoproliferation and inflammatory infiltration in several organs, and die by three weeks of age.
Donating Investigator
Christophe Benoist, Joslin Diabetes Center
Genetic overview
| Genetic Background | Generation |
|---|---|
|
001976 NOD/ShiLtJ |
|
Foxp3sf
| Allele Type | Gene Symbol | Gene Name |
|---|---|---|
| Spontaneous | Foxp3 | forkhead box P3 |
Research Applications
- Diabetes and Obesity Research
- Research Tools
- Cell Biology Research
- Dermatology Research
- Developmental Biology Research
- Endocrine Deficiency Research
- Hematological Research
- Immunology, Inflammation and Autoimmunity Research
Detailed Description
Scurfy mice develop an X-linked lymphoproliferative disease resulting from defective T cell tolerance. By 3 weeks of age, Foxp3-deficient NOD mice suffer massive lymphoproliferation and inflammatory infiltration in lungs, liver, skin, pancreas, kidneys, stomach, colon, fat and muscles and die by three weeks of age. At 14 days of age, NOD.Foxp3-deficient mice developed exocrine pancreatitis and occasional peri-insulitis; however invasive insulitis and diabetes were not observed.
In a NOD.Foxp3-deficient, BDC2.5 TCR transgenic model, mice experienced markedly decreased lymphoproliferation, yet 100% were diabetic by 20 days of age.
This congenic NOD scurfy model is useful to study the role of Foxp3-dependent regulatory T cells on diabetes development.
Development
Foxp3, forkhead box P3, located on the X-Chr. (2.1cM) is necessary for Treg development. An X-linked, spontaneous mutation, Foxp3sf (scurfy) results in loss of function of the Foxp3 gene which leads to a genetic deficit of regulatory T cells. The scurfy mutation arose spontaneously at the Oak Ridge National Laboratory in 1949 in the partially inbred MR stock. This strain was a multiple recessive stock of seven mutations, primarily coat color mutations. Scurfy was maintained either by backcross onto 129/Rl-p Tyrch/p Tyrc or by breeding heterozygous females to (C3H/Rl x 101/Rl)F1 or (101/Rl x C3H/Rl)F1 males at each generation to keep it on a non-inbred background. Means et al. obtained scurfy mice from Yvonne Boyd at Harwell where they were maintained by breeding to (C3H/Rl x 101/Rl)F1. Means et al. backcrossed Foxp3sf/+ females to C57BL/6NTac males. B6.Cg-Foxp3sf mice were backcrossed to NOD. In 2007, the T1DR received this strain at N15 and mated to NOD/ShiLtJ (Stock No. 001976) for 1 generation prior to sibling mating.
Control Suggestions
- Wild-type from the colony
- 001976 NOD/ShiLtJ
Additional Information
Selected References
- Chen Z; Herman AE; Matos M; Mathis D; Benoist C. 2005. Where CD4+CD25+ T reg cells impinge on autoimmune diabetes. J Exp Med 202(10):1387-97. PubMed: 16301745MGI: J:118845
Foxp3sf
Allele Symbol: Foxp3sf
| Allele Name | scurfy |
|---|---|
| Allele Type | Spontaneous |
| Allele Synonym(s) | Scurfy; sf |
| Gene Symbol and Name | Foxp3, forkhead box P3 |
| Gene Synonym(s) | AIID; DIETER; IPEX; JM2; PIDX; RGD1562112; XPID; scurfin; scurfy; sf |
| Strain of Origin | STOCK MR |
| Chromosome | X |
| Molecular Note | Insertion of two adenosine residues into exon 8, resulting in a 2 bp shift in the reading frame. This allele is predicted to produce a truncated protein lacking the carboxy-terminal forkhead domain. |
Disease Terms
Research Areas By Genotype
This mouse can be used to support research in many areas including:
- Diabetes and Obesity Research
- Type 1 Diabetes (IDDM) Analysis Strains
- Research Tools
- Diabetes and Obesity Research
Genotype: Foxp3sf related
- Cell Biology Research
- Transcriptional Regulation
- Dermatology Research
- Skin and Hair Texture Defects
- Developmental Biology Research
- Craniofacial and Palate Defects
- Growth Defects
- Internal/Organ Defects
- gonads
- Lymphoid Tissue Defects
- Postnatal Lethality
- Skin and Hair Texture Defects
- Endocrine Deficiency Research
- Gonad Defects
- Hematological Research
- Anemia, Iron Deficiency and Transport Defects
- Immunological Defects
- Immunology, Inflammation and Autoimmunity Research
- Autoimmunity
- Intracellular Signaling Molecules
- Lymphoid Tissue Defects
- T Cell Receptor Signaling Defects
- Internal/Organ Research
- Lymphoid Tissue Defects
- Reproductive Biology Research
- Developmental Defects Affecting Gonads
- Fertility Defects
References
- Chen Z; Herman AE; Matos M; Mathis D; Benoist C. 2005. Where CD4+CD25+ T reg cells impinge on autoimmune diabetes. J Exp Med 202(10):1387-97. PubMed: 16301745MGI: J:118845
Additional - Foxp3sf related
- Amado IF; Berges J; Luther RJ; Mailhe MP; Garcia S; Bandeira A; Weaver C; Liston A; Freitas AA. 2013. IL-2 coordinates IL-2-producing and regulatory T cell interplay. J Exp Med 210(12):2707-20. PubMed: 24249704MGI: J:208088
- Aschermann S; Lehmann CH; Mihai S; Schett G; Dudziak D; Nimmerjahn F. 2013. B cells are critical for autoimmune pathology in Scurfy mice. Proc Natl Acad Sci U S A 110(47):19042-7. PubMed: 24194550MGI: J:202971
- Bernard JJ; Seweryniak KE; Koniski AD; Spinelli SL; Blumberg N; Francis CW; Taubman MB; Palis J; Phipps RP. 2009. Foxp3 regulates megakaryopoiesis and platelet function. Arterioscler Thromb Vasc Biol 29(11):1874-82. PubMed: 19661482MGI: J:167802
- Beyer M; Thabet Y; Muller RU; Sadlon T; Classen S; Lahl K; Basu S; Zhou X; Bailey-Bucktrout SL; Krebs W; Schonfeld EA; Bottcher J; Golovina T; Mayer CT; Hofmann A; Sommer D; Debey-Pascher S; Endl E; Limmer A; Hippen KL; Blazar BR; Balderas R; Quast T; Waha A; Mayer G; Famulok M; Knolle PA; Wickenhauser C; Kolanus W; Schermer B; Bluestone JA; Barry SC; Sparwasser T; Riley JL; Schultze JL. 2011. Repression of the genome organizer SATB1 in regulatory T cells is required for suppressive function and inhibition of effector differentiation. Nat Immunol 12(9):898-907. PubMed: 21841785MGI: J:176464
- Blair PJ; Bultman SJ; Haas JC; Rouse BT; Wilkinson JE; Godfrey VL. 1994. CD4+CD8- T cells are the effector cells in disease pathogenesis in the scurfy (sf) mouse. J Immunol 153(8):3764-74. PubMed: 7930593MGI: J:20865
- Brunkow ME; Jeffery EW; Hjerrild KA; Paeper B; Clark LB; Yasayko SA; Wilkinson JE; Galas D; Ziegler SF; Ramsdell F. 2001. Disruption of a new forkhead/winged-helix protein, scurfin, results in the fatal lymphoproliferative disorder of the scurfy mouse Nat Genet 27(1):68-73. PubMed: 11138001MGI: J:66695
- Bucher C; Koch L; Vogtenhuber C; Goren E; Munger M; Panoskaltsis-Mortari A; Sivakumar P; Blazar BR. 2009. IL-21 blockade reduces graft-versus-host disease mortality by supporting inducible T regulatory cell generation. Blood 114(26):5375-84. PubMed: 19843883MGI: J:155701
- Burchill MA; Yang J; Vogtenhuber C; Blazar BR; Farrar MA. 2007. IL-2 receptor beta-dependent STAT5 activation is required for the development of Foxp3+ regulatory T cells. J Immunol 178(1):280-90. PubMed: 17182565MGI: J:141932
- Cassani B; Villablanca EJ; Quintana FJ; Love PE; Lacy-Hulbert A; Blaner WS; Sparwasser T; Snapper SB; Weiner HL; Mora JR. 2011. Gut-tropic T cells that express integrin alpha4beta7 and CCR9 are required for induction of oral immune tolerance in mice. Gastroenterology 141(6):2109-18. PubMed: 21925467MGI: J:180414
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- Chang X; Gao JX; Jiang Q; Wen J; Seifers N; Su L; Godfrey VL; Zuo T; Zheng P; Liu Y. 2005. The Scurfy mutation of FoxP3 in the thymus stroma leads to defective thymopoiesis. J Exp Med 202(8):1141-51. PubMed: 16230479MGI: J:116829
- Chang X; Zheng P; Liu Y. 2008. Homeostatic proliferation in the mice with germline FoxP3 mutation and its contribution to fatal autoimmunity. J Immunol 181(4):2399-406. PubMed: 18684929MGI: J:140191
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- He B; Hoang TK; Wang T; Ferris M; Taylor CM; Tian X; Luo M; Tran DQ; Zhou J; Tatevian N; Luo F; Molina JG; Blackburn MR; Gomez TH; Roos S; Rhoads JM; Liu Y. 2017. Resetting microbiota by Lactobacillus reuteri inhibits T reg deficiency-induced autoimmunity via adenosine A2A receptors. J Exp Med 214(1):107-123. PubMed: 27994068MGI: J:238779
- Hirahara K; Ghoreschi K; Yang XP; Takahashi H; Laurence A; Vahedi G; Sciume G; Hall AO; Dupont CD; Francisco LM; Chen Q; Tanaka M; Kanno Y; Sun HW; Sharpe AH; Hunter CA; O'Shea JJ. 2012. Interleukin-27 priming of T cells controls IL-17 production in trans via induction of the ligand PD-L1. Immunity 36(6):1017-30. PubMed: 22726954MGI: J:187415
- Huter EN; Natarajan K; Torgerson TR; Glass DD; Shevach EM. 2010. Autoantibodies in Scurfy mice and IPEX patients recognize keratin 14. J Invest Dermatol 130(5):1391-9. PubMed: 20147963MGI: J:160117
- Huter EN; Punkosdy GA; Glass DD; Cheng LI; Ward JM; Shevach EM. 2008. TGF-beta-induced Foxp3(+) regulatory T cells rescue scurfy mice. Eur J Immunol 38(7):1814-21. PubMed: 18546144MGI: J:137347
- Jang E; Cho WS; Cho ML; Park HJ; Oh HJ; Kang SM; Paik DJ; Youn J. 2011. Foxp3+ regulatory T cells control humoral autoimmunity by suppressing the development of long-lived plasma cells. J Immunol 186(3):1546-53. PubMed: 21209284MGI: J:168900
- Jasurda JS; Jung DO; Froeter ED; Schwartz DB; Hopkins TD; Farris CL; McGee S; Narayan P; Ellsworth BS. 2014. The forkhead transcription factor, FOXP3: a critical role in male fertility in mice. Biol Reprod 90(1):4. PubMed: 24258212MGI: J:210357
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- Kim SV; Xiang WV; Kwak C; Yang Y; Lin XW; Ota M; Sarpel U; Rifkin DB; Xu R; Littman DR. 2013. GPR15-mediated homing controls immune homeostasis in the large intestine mucosa. Science 340(6139):1456-9. PubMed: 23661644MGI: J:199126
- Kleinschnitz C; Kraft P; Dreykluft A; Hagedorn I; Gobel K; Schuhmann MK; Langhauser F; Helluy X; Schwarz T; Bittner S; Mayer CT; Brede M; Varallyay C; Pham M; Bendszus M; Jakob P; Magnus T; Meuth SG; Iwakura Y; Zernecke A; Sparwasser T; Nieswandt B; Stoll G; Wiendl H. 2013. Regulatory T cells are strong promoters of acute ischemic stroke in mice by inducing dysfunction of the cerebral microvasculature. Blood 121(4):679-91. PubMed: 23160472MGI: J:194093
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- Lee JH; Wang C; Kim CH. 2009. FoxP3+ regulatory T cells restrain splenic extramedullary myelopoiesis via suppression of hemopoietic cytokine-producing T cells. J Immunol 183(10):6377-86. PubMed: 19890066MGI: J:157163
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