Strain Name:

NOD.Cg-Foxp3sf/DoiJ

Stock Number:

006775

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Availability:

Cryopreserved - Ready for recovery

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Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain NOD/ShiLtJ
Donor Strain C57BL/6J
H2 Haplotypeg7
GenerationN15+N1F1+N1p
Generation Definitions
 
Donating Investigator Christophe Benoist,   Joslin Diabetes Center

Appearance
pink-eyed, albino
Related Genotype: A/A Tyrc/Tyrc

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 Information

  Control
   Wild-type from the colony
   001976 NOD/ShiLtJ
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Foxp3sf allele
004088   B6.Cg-Foxp3sf/J
View Strains carrying   Foxp3sf     (1 strain)

View Strains carrying other alleles of Foxp3     (16 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Diabetes Mellitus, Insulin-Dependent; IDDM   (FOXP3)
Immunodysregulation, Polyendocrinopathy, and Enteropathy, X-Linked;   (FOXP3)
View Research Applications

Research Applications
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

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

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Foxp3sf
Allele Name scurfy
Allele Type Spontaneous
Common Name(s) Scurfy; sf;
Strain of OriginSTOCK MR
Gene Symbol and Name Foxp3, forkhead box P3
Chromosome X
Gene Common Name(s) AIID; DIETER; IPEX; JM2; PIDX; RGD1562112; XPID; scurfin; scurfy; sf;
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. [MGI Ref ID J:66695]

Genotyping

Genotyping Information

Genotyping Protocols

Foxp3sf, End Point Analysis
Foxp3sf, Pyrosequencing
Foxp3sf, Separated PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

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: 16301745]  [MGI Ref ID J:118845]

Additional References

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: 24249704]  [MGI Ref ID 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: 24194550]  [MGI Ref ID 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: 19661482]  [MGI Ref ID 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: 21841785]  [MGI Ref ID 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: 7930593]  [MGI Ref ID 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: 11138001]  [MGI Ref ID 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: 19843883]  [MGI Ref ID 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: 17182565]  [MGI Ref ID 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: 21925467]  [MGI Ref ID J:180414]

Chang X; Chen L; Wen J; Godfrey VL; Qiao G; Hussien Y; Zhang J; Gao JX. 2006. Foxp3 controls autoreactive T cell activation through transcriptional regulation of early growth response genes and E3 ubiquitin ligase genes, independently of thymic selection. Clin Immunol 121(3):274-85. [PubMed: 16945588]  [MGI Ref ID J:115967]

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: 16230479]  [MGI Ref ID 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: 18684929]  [MGI Ref ID J:140191]

Chen Z; Benoist C; Mathis D. 2005. How defects in central tolerance impinge on a deficiency in regulatory T cells. Proc Natl Acad Sci U S A 102(41):14735-40. [PubMed: 16203996]  [MGI Ref ID J:102496]

Chikuma S; Bluestone JA. 2007. Expression of CTLA-4 and FOXP3 in cis protects from lethal lymphoproliferative disease. Eur J Immunol 37(5):1285-9. [PubMed: 17429849]  [MGI Ref ID J:123580]

Choi JM; Shin JH; Sohn MH; Harding MJ; Park JH; Tobiasova Z; Kim DY; Maher SE; Chae WJ; Park SH; Lee CG; Lee SK; Bothwell AL. 2010. Cell-permeable Foxp3 protein alleviates autoimmune disease associated with inflammatory bowel disease and allergic airway inflammation. Proc Natl Acad Sci U S A 107(43):18575-80. [PubMed: 20937878]  [MGI Ref ID J:165510]

Chung HS; Lee JH; Kim H; Lee HJ; Kim SH; Kwon HK; Im SH; Bae H. 2010. Foxp3 is a novel repressor of microglia activation. Glia 58(10):1247-56. [PubMed: 20544860]  [MGI Ref ID J:168043]

Chung Y; Tanaka S; Chu F; Nurieva RI; Martinez GJ; Rawal S; Wang YH; Lim H; Reynolds JM; Zhou XH; Fan HM; Liu ZM; Neelapu SS; Dong C. 2011. Follicular regulatory T cells expressing Foxp3 and Bcl-6 suppress germinal center reactions. Nat Med 17(8):983-8. [PubMed: 21785430]  [MGI Ref ID J:174509]

Clark LB; Appleby MW; Brunkow ME; Wilkinson JE; Ziegler SF; Ramsdell F. 1999. Cellular and molecular characterization of the scurfy mouse mutant. J Immunol 162(5):2546-54. [PubMed: 10072494]  [MGI Ref ID J:53219]

Curotto de Lafaille MA; Kutchukhidze N; Shen S; Ding Y; Yee H; Lafaille JJ. 2008. Adaptive Foxp3+ regulatory T cell-dependent and -independent control of allergic inflammation. Immunity 29(1):114-26. [PubMed: 18617425]  [MGI Ref ID J:137881]

Derry JM; Wiedemann P; Blair P; Wang Y; Kerns JA; Lemahieu V; Godfrey VL; Wilkinson JE; Francke U. 1995. The mouse homolog of the Wiskott-Aldrich syndrome protein (WASP) gene is highly conserved and maps near the scurfy (sf) mutation on the X chromosome. Genomics 29(2):471-7. [PubMed: 8666397]  [MGI Ref ID J:29221]

Dudda JC; Perdue N; Bachtanian E; Campbell DJ. 2008. Foxp3+ regulatory T cells maintain immune homeostasis in the skin. J Exp Med 205(7):1559-65. [PubMed: 18573908]  [MGI Ref ID J:137389]

Fontenot JD; Gavin MA; Rudensky AY. 2003. Foxp3 programs the development and function of CD4+CD25+ regulatory T cells. Nat Immunol 4(4):330-6. [PubMed: 12612578]  [MGI Ref ID J:82560]

Fousteri G; Jasinski J; Dave A; Nakayama M; Pagni P; Lambolez F; Juntti T; Sarikonda G; Cheng Y; Croft M; Cheroutre H; Eisenbarth G; von Herrath M. 2012. Following the fate of one insulin-reactive CD4 T cell: conversion into Teffs and Tregs in the periphery controls diabetes in NOD mice. Diabetes 61(5):1169-79. [PubMed: 22403296]  [MGI Ref ID J:196727]

Godfrey VL; Rouse BT; Wilkinson JE. 1994. Transplantation of T cell-mediated, lymphoreticular disease from the scurfy (sf) mouse. Am J Pathol 145(2):281-6. [PubMed: 8053488]  [MGI Ref ID J:19699]

Godfrey VL; Wilkinson JE; Russell LB. 1991. X-linked lymphoreticular disease in the scurfy (sf) mutant mouse. Am J Pathol 138(6):1379-87. [PubMed: 2053595]  [MGI Ref ID J:11262]

Gondek DC; Devries V; Nowak EC; Lu LF; Bennett KA; Scott ZA; Noelle RJ. 2008. Transplantation survival is maintained by granzyme B+ regulatory cells and adaptive regulatory T cells. J Immunol 181(7):4752-60. [PubMed: 18802078]  [MGI Ref ID J:141831]

Granville CA; Memmott RM; Balogh A; Mariotti J; Kawabata S; Han W; Lopiccolo J; Foley J; Liewehr DJ; Steinberg SM; Fowler DH; Hollander MC; Dennis PA. 2009. A central role for Foxp3+ regulatory T cells in K-Ras-driven lung tumorigenesis. PLoS ONE 4(3):e5061. [PubMed: 19330036]  [MGI Ref ID J:147455]

Gratz IK; Rosenblum MD; Maurano MM; Paw JS; Truong HA; Marshak-Rothstein A; Abbas AK. 2014. Cutting edge: Self-antigen controls the balance between effector and regulatory T cells in peripheral tissues. J Immunol 192(4):1351-5. [PubMed: 24442443]  [MGI Ref ID J:209358]

Guo L; Tian J; Marinova E; Zheng B; Han S. 2010. Inhibition of clonal expansion by Foxp3 expression as a mechanism of controlled T-cell responses and autoimmune disease. Eur J Immunol 40(1):71-80. [PubMed: 19877010]  [MGI Ref ID J:155697]

Han KL; Thomas SV; Koontz SM; Changpriroa CM; Ha SK; Malech HL; Kang EM. 2013. Adenosine A2A Receptor Agonist-Mediated Increase in Donor-Derived Regulatory T Cells Suppresses Development of Graft-versus-Host Disease. J Immunol 190(1):458-68. [PubMed: 23225892]  [MGI Ref ID J:190811]

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: 22726954]  [MGI Ref ID 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: 20147963]  [MGI Ref ID 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: 18546144]  [MGI Ref ID 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: 21209284]  [MGI Ref ID 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: 24258212]  [MGI Ref ID J:210357]

Jung DO; Jasurda JS; Egashira N; Ellsworth BS. 2012. The forkhead transcription factor, FOXP3, is required for normal pituitary gonadotropin expression in mice. Biol Reprod 86(5):144, 1-9. [PubMed: 22357547]  [MGI Ref ID J:185832]

Kanangat S; Blair P; Reddy R; Deheshia M; Godfrey V; Rouse BT; Wilkinson E. 1996. Disease in the scurfy (sf) mouse is associated with overexpression of cytokine genes. Eur J Immunol 26(1):161-5. [PubMed: 8566060]  [MGI Ref ID J:33090]

Khattri R; Cox T; Yasayko SA; Ramsdell F. 2003. An essential role for Scurfin in CD4+CD25+ T regulatory cells. Nat Immunol 4(4):337-42. [PubMed: 12612581]  [MGI Ref ID J:128566]

Khattri R; Kasprowicz D; Cox T; Mortrud M; Appleby MW; Brunkow ME; Ziegler SF; Ramsdell F. 2001. The amount of scurfin protein determines peripheral T cell number and responsiveness. J Immunol 167(11):6312-20. [PubMed: 11714795]  [MGI Ref ID J:72828]

Kim CH. 2006. Migration and function of FoxP3+ regulatory T cells in the hematolymphoid system. Exp Hematol 34(8):1033-40. [PubMed: 16863909]  [MGI Ref ID J:111905]

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: 23661644]  [MGI Ref ID 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: 23160472]  [MGI Ref ID J:194093]

Koch MA; Tucker-Heard G; Perdue NR; Killebrew JR; Urdahl KB; Campbell DJ. 2009. The transcription factor T-bet controls regulatory T cell homeostasis and function during type 1 inflammation. Nat Immunol 10(6):595-602. [PubMed: 19412181]  [MGI Ref ID J:149556]

Koenecke C; Lee CW; Thamm K; Fohse L; Schafferus M; Mittrucker HW; Floess S; Huehn J; Ganser A; Forster R; Prinz I. 2012. IFN-gamma production by allogeneic Foxp3+ regulatory T cells is essential for preventing experimental graft-versus-host disease. J Immunol 189(6):2890-6. [PubMed: 22869903]  [MGI Ref ID J:189843]

Komatsu N; Hori S. 2007. Full restoration of peripheral Foxp3+ regulatory T cell pool by radioresistant host cells in scurfy bone marrow chimeras. Proc Natl Acad Sci U S A 104(21):8959-64. [PubMed: 17494743]  [MGI Ref ID J:121851]

Kuczma M; Lee JR; Kraj P. 2011. Connexin 43 signaling enhances the generation of Foxp3+ regulatory T cells. J Immunol 187(1):248-57. [PubMed: 21642545]  [MGI Ref ID J:176183]

Kuczma M; Podolsky R; Garge N; Daniely D; Pacholczyk R; Ignatowicz L; Kraj P. 2009. Foxp3-deficient regulatory T cells do not revert into conventional effector CD4+ T cells but constitute a unique cell subset. J Immunol 183(6):3731-41. [PubMed: 19710455]  [MGI Ref ID J:152306]

Lahl K; Loddenkemper C; Drouin C; Freyer J; Arnason J; Eberl G; Hamann A; Wagner H; Huehn J; Sparwasser T. 2007. Selective depletion of Foxp3+ regulatory T cells induces a scurfy-like disease. J Exp Med 204(1):57-63. [PubMed: 17200412]  [MGI Ref ID J:125295]

Lahl K; Mayer CT; Bopp T; Huehn J; Loddenkemper C; Eberl G; Wirnsberger G; Dornmair K; Geffers R; Schmitt E; Buer J; Sparwasser T. 2009. Nonfunctional regulatory T cells and defective control of Th2 cytokine production in natural scurfy mutant mice. J Immunol 183(9):5662-72. [PubMed: 19812199]  [MGI Ref ID J:156808]

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: 19890066]  [MGI Ref ID J:157163]

Lee K; Hwang S; Paik DJ; Kim WK; Kim JM; Youn J. 2012. Bacillus-derived poly-gamma-glutamic acid reciprocally regulates the differentiation of T helper 17 and regulatory T cells and attenuates experimental autoimmune encephalomyelitis. Clin Exp Immunol 170(1):66-76. [PubMed: 22943202]  [MGI Ref ID J:188286]

Leung MW; Shen S; Lafaille JJ. 2009. TCR-dependent differentiation of thymic Foxp3+ cells is limited to small clonal sizes. J Exp Med 206(10):2121-30. [PubMed: 19737865]  [MGI Ref ID J:153359]

Liston A; Farr AG; Chen Z; Benoist C; Mathis D; Manley NR; Rudensky AY. 2007. Lack of Foxp3 function and expression in the thymic epithelium. J Exp Med 204(3):475-80. [PubMed: 17353370]  [MGI Ref ID J:125405]

Liu G; Burns S; Huang G; Boyd K; Proia RL; Flavell RA; Chi H. 2009. The receptor S1P1 overrides regulatory T cell-mediated immune suppression through Akt-mTOR. Nat Immunol 10(7):769-77. [PubMed: 19483717]  [MGI Ref ID J:150139]

Liu G; Yang K; Burns S; Shrestha S; Chi H. 2010. The S1P(1)-mTOR axis directs the reciprocal differentiation of T(H)1 and T(reg) cells. Nat Immunol 11(11):1047-56. [PubMed: 20852647]  [MGI Ref ID J:166555]

Liu R; Wang L; Chen G; Katoh H; Chen C; Liu Y; Zheng P. 2009. FOXP3 up-regulates p21 expression by site-specific inhibition of histone deacetylase 2/histone deacetylase 4 association to the locus. Cancer Res 69(6):2252-9. [PubMed: 19276356]  [MGI Ref ID J:147076]

Lochner M; Peduto L; Cherrier M; Sawa S; Langa F; Varona R; Riethmacher D; Si-Tahar M; Di Santo JP; Eberl G. 2008. In vivo equilibrium of proinflammatory IL-17+ and regulatory IL-10+ Foxp3+ RORgamma t+ T cells. J Exp Med 205(6):1381-93. [PubMed: 18504307]  [MGI Ref ID J:137020]

Lu Y; Suzuki J; Guillioli M; Umland O; Chen Z. 2011. Induction of self-antigen-specific Foxp3+ regulatory T cells in the periphery by lymphodepletion treatment with anti-mouse thymocyte globulin in mice. Immunology 134(1):50-9. [PubMed: 21711461]  [MGI Ref ID J:176176]

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Lyon MF; Peters J; Glenister PH; Ball S; Wright E. 1990. The scurfy mouse mutant has previously unrecognized hematological abnormalities and resembles Wiskott-Aldrich syndrome. Proc Natl Acad Sci U S A 87(7):2433-7. [PubMed: 2320565]  [MGI Ref ID J:10398]

Miska J; Bas E; Devarajan P; Chen Z. 2012. Autoimmunity-mediated antitumor immunity: Tumor as an immunoprivileged self. Eur J Immunol 42(10):2584-96. [PubMed: 22777737]  [MGI Ref ID J:188006]

Murray SE; Polesso F; Rowe AM; Basak S; Koguchi Y; Toren KG; Hoffmann A; Parker DC. 2011. NF-kappaB-inducing kinase plays an essential T cell-intrinsic role in graft-versus-host disease and lethal autoimmunity in mice. J Clin Invest 121(12):4775-86. [PubMed: 22045568]  [MGI Ref ID J:184029]

Nakagawa T; Tsuruoka M; Ogura H; Okuyama Y; Arima Y; Hirano T; Murakami M. 2010. IL-6 positively regulates Foxp3+CD8+ T cells in vivo. Int Immunol 22(2):129-39. [PubMed: 20042455]  [MGI Ref ID J:157654]

Nishimura H; Strominger JL. 2006. Involvement of a tissue-specific autoantibody in skin disorders of murine systemic lupus erythematosus and autoinflammatory diseases. Proc Natl Acad Sci U S A 103(9):3292-7. [PubMed: 16492738]  [MGI Ref ID J:107174]

Oliveira VG; Caridade M; Paiva RS; Demengeot J; Graca L. 2011. Sub-optimal CD4+ T-cell activation triggers autonomous TGF-beta-dependent conversion to Foxp3+ regulatory T cells. Eur J Immunol 41(5):1249-55. [PubMed: 21469093]  [MGI Ref ID J:175409]

Ouyang W; Beckett O; Ma Q; Paik JH; DePinho RA; Li MO. 2010. Foxo proteins cooperatively control the differentiation of Foxp3+ regulatory T cells. Nat Immunol 11(7):618-27. [PubMed: 20467422]  [MGI Ref ID J:161859]

Ouyang W; Liao W; Luo CT; Yin N; Huse M; Kim MV; Peng M; Chan P; Ma Q; Mo Y; Meijer D; Zhao K; Rudensky AY; Atwal G; Zhang MQ; Li MO. 2012. Novel Foxo1-dependent transcriptional programs control T(reg) cell function. Nature 491(7425):554-9. [PubMed: 23135404]  [MGI Ref ID J:189962]

Pandiyan P; Zheng L; Ishihara S; Reed J; Lenardo MJ. 2007. CD4(+)CD25(+)Foxp3(+) regulatory T cells induce cytokine deprivation-mediated apoptosis of effector CD4(+) T cells. Nat Immunol 8(12):1353-62. [PubMed: 17982458]  [MGI Ref ID J:127761]

Patel DD. 2001. Escape from tolerance in the human X-linked autoimmunity-allergic disregulation syndrome and the Scurfy mouse. J Clin Invest 107(2):155-7. [PubMed: 11160129]  [MGI Ref ID J:66976]

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Ramsdell F; Peake J; Faravelli F; Casanova JL; Buist N; Levy-Lahad E; Mazzella M; Goulet O; Perroni L; Dagna Bricarelli F; Byrne G; McEuen M; Proll S; Appleby M; Brunkow ME; Wildin RS. 2001. X-linked neonatal diabetes mellitus, enteropathy and endocrinopathy syndrome is the human equivalent of mouse scurfy Nat Genet 27(1):18-20. [PubMed: 11137992]  [MGI Ref ID J:66734]

Romagnoli P; Dooley J; Enault G; Vicente R; Malissen B; Liston A; van Meerwijk JP. 2012. The thymic niche does not limit development of the naturally diverse population of mouse regulatory T lymphocytes. J Immunol 189(8):3831-7. [PubMed: 22988035]  [MGI Ref ID J:190638]

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Sanchez G; Dury AY; Murray LM; Biondi O; Tadesse H; El Fatimy R; Kothary R; Charbonnier F; Khandjian EW; Cote J. 2013. A novel function for the survival motoneuron protein as a translational regulator. Hum Mol Genet 22(4):668-84. [PubMed: 23136128]  [MGI Ref ID J:191211]

Sharma R; Deshmukh US; Zheng L; Fu SM; Ju ST. 2009. X-linked Foxp3 (Scurfy) mutation dominantly inhibits submandibular gland development and inflammation respectively through adaptive and innate immune mechanisms. J Immunol 183(5):3212-8. [PubMed: 19648271]  [MGI Ref ID J:151870]

Sharma R; Jarjour WN; Zheng L; Gaskin F; Fu SM; Ju ST. 2007. Large functional repertoire of regulatory T-cell suppressible autoimmune T cells in scurfy mice. J Autoimmun 29(1):10-9. [PubMed: 17521882]  [MGI Ref ID J:125102]

Sharma R; Ju AC; Kung JT; Fu SM; Ju ST. 2008. Rapid and selective expansion of nonclonotypic T cells in regulatory T cell-deficient, foreign antigen-specific TCR-transgenic scurfy mice: antigen-dependent expansion and TCR analysis. J Immunol 181(10):6934-41. [PubMed: 18981113]  [MGI Ref ID J:140943]

Sharma R; Sharma PR; Kim YC; Leitinger N; Lee JK; Fu SM; Ju ST. 2011. IL-2-controlled expression of multiple T cell trafficking genes and Th2 cytokines in the regulatory T cell-deficient scurfy mice: implication to multiorgan inflammation and control of skin and lung inflammation. J Immunol 186(2):1268-78. [PubMed: 21169543]  [MGI Ref ID J:168761]

Sharma R; Sung SS; Abaya CE; Ju AC; Fu SM; Ju ST. 2009. IL-2 regulates CD103 expression on CD4+ T cells in Scurfy mice that display both CD103-dependent and independent inflammation. J Immunol 183(2):1065-73. [PubMed: 19553521]  [MGI Ref ID J:151408]

Sharma R; Zheng L; Deshmukh US; Jarjour WN; Sung SS; Fu SM; Ju ST. 2007. A regulatory T cell-dependent novel function of CD25 (IL-2Ralpha) controlling memory CD8(+) T cell homeostasis. J Immunol 178(3):1251-5. [PubMed: 17237369]  [MGI Ref ID J:143666]

Sharma R; Zheng L; Guo X; Fu SM; Ju ST; Jarjour WN. 2006. Novel animal models for Sjogren's syndrome: expression and transfer of salivary gland dysfunction from regulatory T cell-deficient mice. J Autoimmun 27(4):289-96. [PubMed: 17207605]  [MGI Ref ID J:125129]

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Thornton AM; Korty PE; Tran DQ; Wohlfert EA; Murray PE; Belkaid Y; Shevach EM. 2010. Expression of Helios, an Ikaros transcription factor family member, differentiates thymic-derived from peripherally induced Foxp3+ T regulatory cells. J Immunol 184(7):3433-41. [PubMed: 20181882]  [MGI Ref ID J:160098]

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Yamaguchi T; Kishi A; Osaki M; Morikawa H; Prieto-Martin P; Wing K; Saito T; Sakaguchi S. 2013. Construction of self-recognizing regulatory T cells from conventional T cells by controlling CTLA-4 and IL-2 expression. Proc Natl Acad Sci U S A 110(23):E2116-25. [PubMed: 23690575]  [MGI Ref ID J:197416]

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Health & husbandry

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Breeding & HusbandryOnly the heterozygous female will be available for distribution, the hemizygous male dies too early to be shipped. Breeder pair is het female x wildtype or NOD/ShiLtJ (Stock No. 001976) male (not reciprocal). Affected mutant is hemizygous male. Homozygous female would also be affected, but traditional breeding schemes will not allow for the female to be born.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2525.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Frozen Products

Price (US dollars $)
Frozen Embryo $1650.00

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3283.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Frozen Products

Price (US dollars $)
Frozen Embryo $2145.00

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

General Supply Notes

Control Information

  Control
   Wild-type from the colony
   001976 NOD/ShiLtJ
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
Ordering Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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