Strain Name:

B6.Cg-Foxp3sf/J

Stock Number:

004088

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Scurfy mice have defective T cell tolerance leading to an X-linked lymphoproliferative disease that parallels the X-linked autoimmunity-allergic disregulation syndrome (XLAAD) in humans. Hemizygous males are characterized by runting, scaly, crusty skin on the eyelids, ears and tails, dermal thickening, squinted eyes, cachexia, reddening and swelling of the genital papilla, and small testicles that are retained in the abdominal cavity. Homozygous scurfy females develop the same disease phenotype seen in hemizygous males, but they have a normal reproductive tract.

Description

Strain Information

Former Names B.Cg-Foxp3sf    (Changed: 15-DEC-04 )
Type Congenic; Mutant Strain;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Mating SystemHeterozygote x Inbred         (Female x Male)   01-MAR-06
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain Mixed
GenerationN8+N31 (18-OCT-13)
Generation Definitions

Description
Scurfy mice develop an X-linked lymphoproliferative disease resulting from defective T cell tolerance. Phenotypes associated with these mice include runting, scaly, crusty skin on the eyelids, ears and tails, dermal thickening, squinted eyes, cachexia, reddening and swelling of the genital papilla, and small testicles that are retained in the abdominal cavity. This disorder, which parallels X-linked autoimmunity-allergic disregulation syndrome (XLAAD) in humans, results in Coombs' test-positive anemia, hypergammaglobulinemia, a small, thin thymus, and lymphohistiocytic proliferation in the skin and lymphoid organs, with splenomegaly, lymphadenomegaly, and hepatomegaly. Foxp3sf/Y males generally die by 16-25 days of age. Transgenic expression of Foxp3 prevents scurfy disease in Foxp3sf/Y mice.

Neonatal thymectomy of scurfy males ameliorates disease and increases lifespan; athymic nude (Foxn1nu/Foxn1nu) Foxp3sf/Y mice do not develop scurfy. While Cd4+ peripheral T cells from scurfy mice can transfer the scurfy disease phenotype to wild type, histocompatible Foxn1nu/Foxn1nu or Prkdcscid/Prkdcscid hosts, bone marrow transplantation from scurfy homozygotes fails to transfer disease. Also, neither neonatal inoculation with wild type bone marrow, nor thymic lobe transplants from wild type donors into carrier males prevents disease. Northern blot analysis of skin, lymph nodes and spleen revealed over-expression of Il2, Il4, Il5, Il10, Il6, IFNg, and TNFa; over-expression of these last three is especially high. Peripheral Cd4+ T cells from scurfy mice are hyper-responsive to antigen, have an activated phenotype (Cd44+, Cd69+, Cd25+, Cd80+, Cd86+), a decreased requirement for Cd28 co-stimulation, and a decreased sensitivity to tyrosine kinase inhibitors and cyclosporin A. Prenatal or neonatal injection with anti-Cd4 antibodies can delay the onset of disease, as can the targeted disruption of Cd4. Cd8+ cells do not transfer disease, and targeted disruption of B2m does not alter disease onset. Activation of peripheral T cells is necessary to initiate the scurfy pathology; Foxp3sf/Y mice carrying a transgene for an ovalbumin-specific TCR and a targeted mutation of Rag1 fail to develop the scurfy disease phenotype until challenged with ovalbumin. Foxp3sf homozygous females can not be generated through traditional breeding because carrier males die by 25 days of age. By breeding nude Foxp3sf/Y males with Foxp3sf/+ females, however, homozygous scurfy females can be generated that are heterozygous for the recessive Foxn1nu mutation. These homozygous scurfy females develop the same disease phenotype seen in hemizygous males, but they have a normal reproductive tract.

Development
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. In 2001 The Jackson Laboratory received N8 mice and backcrossed to C57BL/6J. (Russell et al., 1959; Godfrey et al., 1991; Means et al., 2000.)

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Foxp3sf allele
006775   NOD.Cg-Foxp3sf/DoiJ
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
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Immunodysregulation, Polyendocrinopathy, and Enteropathy, X-Linked;
Models with phenotypic similarity to human diseases where etiology is unknown or involving genes where ortholog is unknown.
Ichthyosis and Male Hypogonadism
Wiskott-Aldrich Syndrome; WAS
- No similarity to the expected human disease phenotype was found. One or more human genes are associated with this human disease. The mouse genotype may involve mutations to orthologs of one or more of these genes, but the phenotype did not resemble the disease.
Ichthyosis, X-Linked; XLI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Diabetes Mellitus, Insulin-Dependent; IDDM   (FOXP3)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Foxp3sf/Y

        B6.Cg-Foxp3sf
  • hematopoietic system phenotype
  • abnormal CD4-positive T cell morphology   (MGI Ref ID J:82560)
    • decreased CD4-positive, CD25-positive, alpha-beta regulatory T cell number
      • at 10 days of age there are significantly fewer CD4+ CD25+ T cells in the lymph nodes and thymus compared with controls and transfer of CD4+ CD25+ T cells from wild-type donors into 1- to 2-day old hemizygous pups rescues the disease phenotype   (MGI Ref ID J:82560)
  • abnormal CD4-positive T cell physiology
    • CD4+ CD25+ T cells from 28 day old lymph nodes proliferate abnormally in response to TCR stimulation and fail to provide suppressor activity   (MGI Ref ID J:82560)
    • transfer of CD4+ T cells from hemizygotes into RAG1 deficient hosts transfers the wasting disease and colitis and co-transfer of CD4+ CD25+ from wild-type donors prevents the development of this disease   (MGI Ref ID J:82560)
  • abnormal megakaryocyte progenitor cell morphology
    • mutants exhibit about a 50% reduction in megakaryocyte progenitors   (MGI Ref ID J:167802)
    • decreased megakaryocyte cell number
      • mutants exhibit about 4-fold less mature bone marrow megakaryocytes than controls   (MGI Ref ID J:167802)
    • decreased platelet cell number
      • mutants exhibit up to 53% fewer platelets than controls   (MGI Ref ID J:167802)
    • increased mean platelet volume   (MGI Ref ID J:167802)
  • homeostasis/metabolism phenotype
  • abnormal homeostasis
    • mutants exhibit reduced serum levels of TGF-beta and increased serum levels of CD40L, TXB2, and 12(S)-HETE, suggesting altered platelet release   (MGI Ref ID J:167802)
  • immune system phenotype
  • abnormal CD4-positive T cell morphology   (MGI Ref ID J:82560)
    • decreased CD4-positive, CD25-positive, alpha-beta regulatory T cell number
      • at 10 days of age there are significantly fewer CD4+ CD25+ T cells in the lymph nodes and thymus compared with controls and transfer of CD4+ CD25+ T cells from wild-type donors into 1- to 2-day old hemizygous pups rescues the disease phenotype   (MGI Ref ID J:82560)
  • abnormal CD4-positive T cell physiology
    • CD4+ CD25+ T cells from 28 day old lymph nodes proliferate abnormally in response to TCR stimulation and fail to provide suppressor activity   (MGI Ref ID J:82560)
    • transfer of CD4+ T cells from hemizygotes into RAG1 deficient hosts transfers the wasting disease and colitis and co-transfer of CD4+ CD25+ from wild-type donors prevents the development of this disease   (MGI Ref ID J:82560)

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Foxp3sf/Y

        involves: STOCK MR
  • mortality/aging
  • postnatal lethality
    • female mice that only have one X chromosome that carries this mutation die before being able to reproduce   (MGI Ref ID J:13126)
  • vision/eye phenotype
  • delayed eyelid opening
    • eyelids are delayed in opening   (MGI Ref ID J:13126)
  • integument phenotype
  • scaly skin
    • first the tail, and then other parts of the body exhibit scaliness   (MGI Ref ID J:13126)
  • tight skin
    • mice also have tight skin   (MGI Ref ID J:13126)

Foxp3sf/Y

        involves: STOCK MR
  • mortality/aging
  • postnatal lethality
    • 2/3 die before weaning and most of the rest shortly after weaning, though occasionally can live for several months   (MGI Ref ID J:13126)
  • premature death
    • the vast majority of mice that live past weaning die shortly thereafter   (MGI Ref ID J:13126)
  • growth/size/body phenotype
  • decreased body size
    • the few mice that live to adulthood are runty   (MGI Ref ID J:13126)
  • reproductive system phenotype
  • abnormal male reproductive system morphology
    • reddening of the genital papilla at P11   (MGI Ref ID J:13126)
    • absent prostate gland anterior lobe   (MGI Ref ID J:14076)
    • absent scrotum   (MGI Ref ID J:14076)
    • absent seminal vesicle   (MGI Ref ID J:14076)
    • cryptorchism
      • testes are abdominal   (MGI Ref ID J:14076)
    • small testis   (MGI Ref ID J:14076)
  • male infertility   (MGI Ref ID J:14076)
    • the few mice that live to adulthood are infertile   (MGI Ref ID J:13126)
  • small gonad   (MGI Ref ID J:14076)
    • small testis   (MGI Ref ID J:14076)
  • vision/eye phenotype
  • delayed eyelid opening
    • eyelids are delayed in opening   (MGI Ref ID J:13126)
  • endocrine/exocrine gland phenotype
  • absent prostate gland anterior lobe   (MGI Ref ID J:14076)
  • absent seminal vesicle   (MGI Ref ID J:14076)
  • cryptorchism
    • testes are abdominal   (MGI Ref ID J:14076)
  • small testis   (MGI Ref ID J:14076)
  • integument phenotype
  • scaly skin
    • exhibit ichythosis   (MGI Ref ID J:14076)
    • first the tail, and then other parts of the body exhibit scaliness   (MGI Ref ID J:13126)
  • tight skin
    • mice also have tight skin   (MGI Ref ID J:13126)

Foxp3sf/Y

        either: 129Rl.Cg-Foxp3sf or (involves: 101/Rl * C3Hf/Rl * STOCK MR)
  • mortality/aging
  • premature death
    • mean lifespan is about 24 days although a few survive to 30-39 days of age   (MGI Ref ID J:11262)
  • growth/size/body phenotype
  • decreased body size   (MGI Ref ID J:11262)
  • distended abdomen
    • swollen abdomen   (MGI Ref ID J:11262)
  • scaly ears
    • crusting of the ears by 14-15 days of age   (MGI Ref ID J:11262)
  • small ears
    • ears are small and sometimes folded   (MGI Ref ID J:11262)
  • thick ears   (MGI Ref ID J:11262)
  • vision/eye phenotype
  • blepharitis
    • scaliness on eyelids that seals the palpebral fissure   (MGI Ref ID J:11262)
  • hearing/vestibular/ear phenotype
  • scaly ears
    • crusting of the ears by 14-15 days of age   (MGI Ref ID J:11262)
  • small ears
    • ears are small and sometimes folded   (MGI Ref ID J:11262)
  • thick ears   (MGI Ref ID J:11262)
  • reproductive system phenotype
  • abnormal male reproductive system morphology
    • swelling and reddening of the genital papilla at 12-14 days of age   (MGI Ref ID J:11262)
    • cryptorchism
      • testicles are retained in the abdominal cavity   (MGI Ref ID J:11262)
    • small testis   (MGI Ref ID J:11262)
  • immune system phenotype
  • abnormal B cell physiology
    • IgA synthesis is precocious, detectable at P21 unlike in controls   (MGI Ref ID J:11262)
    • increased IgG level
      • apparent as early as 10 days of age and ranges from 2-10 times the concentration in controls   (MGI Ref ID J:11262)
    • increased IgM level   (MGI Ref ID J:11262)
  • abnormal lymph node morphology
    • lesions in the lymph nodes that contain a randomly distributed mixture of lymphoblasts, blastlike mononuclear cells with vesicular nuclei, hypertrophic reticulum cells, macrophages, and granulocytes instead of small lymphocytes   (MGI Ref ID J:11262)
    • complete loss of normal architecture   (MGI Ref ID J:11262)
    • abnormal lymph node B cell domain morphology
      • lack of discernible follicles   (MGI Ref ID J:11262)
    • abnormal lymph node T cell domain morphology
      • no distinct paracortex   (MGI Ref ID J:11262)
    • abnormal lymph node medullary cord morphology
      • thickening of the medullary cords   (MGI Ref ID J:11262)
    • enlarged lymph nodes
      • subcutaneous lymph nodes such as inguinal, axillary, and cervical nodes and consistently enlarged while visceral lymph nodes are slightly or moderately enlarged   (MGI Ref ID J:11262)
  • abnormal lymphocyte morphology
    • exhibit lymphoproliferative lesions   (MGI Ref ID J:11262)
  • abnormal macrophage morphology
    • mild to moderate sinus histiocytosis in the lymph nodes   (MGI Ref ID J:11262)
  • abnormal spleen morphology
    • lesions in the spleen   (MGI Ref ID J:11262)
    • abnormal spleen white pulp morphology
      • white pulp may be enlarged or shrunken and is composed of blastlike mononuclear cells with vesicular nuclei, prominent reticulum cells, lymphoblasts, and variable number of plasma cells   (MGI Ref ID J:11262)
      • occasional erythrophagocytic macrophages and plasma cells with Russel's bodies can be seen at the margins of the white pulp   (MGI Ref ID J:11262)
      • abnormal spleen B cell follicle morphology
        • follicles are lacking and lymphocytes are absent in the spleen   (MGI Ref ID J:11262)
      • absent spleen marginal zone
        • distinct marginal zones are lacking   (MGI Ref ID J:11262)
    • increased spleen red pulp amount
      • massively expanded by hematopoietic cells   (MGI Ref ID J:11262)
    • increased spleen weight
      • spleen weights are 2-4 times those of controls   (MGI Ref ID J:11262)
  • abnormal thymus morphology   (MGI Ref ID J:11262)
    • abnormal thymus cortex morphology
      • the thymic cortex is rapidly depleted of lymphocytes over time   (MGI Ref ID J:11262)
    • small thymus
      • a bilobed thymus is present but extremely small and is densely populated with lymphocytes   (MGI Ref ID J:11262)
  • increased inflammatory response
    • perivascular infiltrates of mixed mononuclear cells and granulocytes are seen in heart, pancreas, lung, salivary gland, kidney, and mesenteries   (MGI Ref ID J:11262)
    • blepharitis
      • scaliness on eyelids that seals the palpebral fissure   (MGI Ref ID J:11262)
    • dermatitis
      • a diffuse lymphohistiocytic infiltration of the entire dermis that is most severe in the prepuce, ears, eyelids and facial skin   (MGI Ref ID J:11262)
    • liver inflammation
      • leukocytic infiltrations are present in the portal areas of the liver   (MGI Ref ID J:11262)
  • increased leukocyte cell number   (MGI Ref ID J:11262)
  • hematopoietic system phenotype
  • abnormal B cell physiology
    • IgA synthesis is precocious, detectable at P21 unlike in controls   (MGI Ref ID J:11262)
    • increased IgG level
      • apparent as early as 10 days of age and ranges from 2-10 times the concentration in controls   (MGI Ref ID J:11262)
    • increased IgM level   (MGI Ref ID J:11262)
  • abnormal erythrocyte morphology   (MGI Ref ID J:11262)
    • anemia   (MGI Ref ID J:11262)
    • anisocytosis   (MGI Ref ID J:11262)
    • decreased hematocrit
      • mean hematocrit values are about one half those of controls   (MGI Ref ID J:11262)
    • decreased hemoglobin content
      • mean hemoglobin volumes are about one half those of controls   (MGI Ref ID J:11262)
    • increased mean corpuscular volume   (MGI Ref ID J:11262)
    • poikilocytosis   (MGI Ref ID J:11262)
    • polychromatophilia   (MGI Ref ID J:11262)
  • abnormal lymphocyte morphology
    • exhibit lymphoproliferative lesions   (MGI Ref ID J:11262)
  • abnormal macrophage morphology
    • mild to moderate sinus histiocytosis in the lymph nodes   (MGI Ref ID J:11262)
  • abnormal spleen morphology
    • lesions in the spleen   (MGI Ref ID J:11262)
    • abnormal spleen white pulp morphology
      • white pulp may be enlarged or shrunken and is composed of blastlike mononuclear cells with vesicular nuclei, prominent reticulum cells, lymphoblasts, and variable number of plasma cells   (MGI Ref ID J:11262)
      • occasional erythrophagocytic macrophages and plasma cells with Russel's bodies can be seen at the margins of the white pulp   (MGI Ref ID J:11262)
      • abnormal spleen B cell follicle morphology
        • follicles are lacking and lymphocytes are absent in the spleen   (MGI Ref ID J:11262)
      • absent spleen marginal zone
        • distinct marginal zones are lacking   (MGI Ref ID J:11262)
    • increased spleen red pulp amount
      • massively expanded by hematopoietic cells   (MGI Ref ID J:11262)
    • increased spleen weight
      • spleen weights are 2-4 times those of controls   (MGI Ref ID J:11262)
  • abnormal thymus morphology   (MGI Ref ID J:11262)
    • abnormal thymus cortex morphology
      • the thymic cortex is rapidly depleted of lymphocytes over time   (MGI Ref ID J:11262)
    • small thymus
      • a bilobed thymus is present but extremely small and is densely populated with lymphocytes   (MGI Ref ID J:11262)
  • extramedullary hematopoiesis
    • abundant hematopoiesis in the hepatic sinusoids, spleen and bone marrow   (MGI Ref ID J:11262)
  • increased leukocyte cell number   (MGI Ref ID J:11262)
  • liver/biliary system phenotype
  • abnormal liver morphology
    • lesions in the liver   (MGI Ref ID J:11262)
    • occasionally livers have marked erythrophagocytosis or hemosiderin deposits in Kupffer cells   (MGI Ref ID J:11262)
    • abnormal hepatic cord morphology
      • centrolobular hepatic cords are atrophied   (MGI Ref ID J:11262)
    • enlarged liver sinusoidal spaces   (MGI Ref ID J:11262)
    • enlarged liver   (MGI Ref ID J:11262)
    • multifocal hepatic necrosis
      • many mice have a thin, sharply demarcated rim of necrosis along the margins of the liver   (MGI Ref ID J:11262)
      • areas of acute coagulative necrosis without inflammation are present at the tips of liver lobes   (MGI Ref ID J:11262)
  • jaundice
    • severely anemic mice may exhibit slight icterus   (MGI Ref ID J:11262)
  • liver inflammation
    • leukocytic infiltrations are present in the portal areas of the liver   (MGI Ref ID J:11262)
  • homeostasis/metabolism phenotype
  • atrial thrombosis
    • occasionally see acute right atrial thrombosis   (MGI Ref ID J:11262)
  • hemosiderosis
    • occasionally livers have marked hemosiderin deposits in Kupffer cells   (MGI Ref ID J:11262)
  • pleural effusion
    • severely anemic mice may exhibit pleural effusion   (MGI Ref ID J:11262)
  • renal/urinary system phenotype
  • pale kidney   (MGI Ref ID J:11262)
  • behavior/neurological phenotype
  • hunched posture   (MGI Ref ID J:11262)
  • cardiovascular system phenotype
  • enlarged heart
    • severely anemic mice may exhibit cardiomegaly   (MGI Ref ID J:11262)
  • enlarged liver sinusoidal spaces   (MGI Ref ID J:11262)
  • endocrine/exocrine gland phenotype
  • abnormal thymus morphology   (MGI Ref ID J:11262)
    • abnormal thymus cortex morphology
      • the thymic cortex is rapidly depleted of lymphocytes over time   (MGI Ref ID J:11262)
    • small thymus
      • a bilobed thymus is present but extremely small and is densely populated with lymphocytes   (MGI Ref ID J:11262)
  • cryptorchism
    • testicles are retained in the abdominal cavity   (MGI Ref ID J:11262)
  • small testis   (MGI Ref ID J:11262)
  • craniofacial phenotype
  • scaly ears
    • crusting of the ears by 14-15 days of age   (MGI Ref ID J:11262)
  • small ears
    • ears are small and sometimes folded   (MGI Ref ID J:11262)
  • thick ears   (MGI Ref ID J:11262)
  • integument phenotype
  • abnormal epidermal layer morphology
    • occasionally see intraepidermal pustules   (MGI Ref ID J:11262)
    • epidermal hyperplasia   (MGI Ref ID J:11262)
    • orthokeratosis
      • moderate to severe orthokeratotic hyperkeratosis   (MGI Ref ID J:11262)
    • parakeratosis
      • multifocal parakeratosis   (MGI Ref ID J:11262)
  • dermatitis
    • a diffuse lymphohistiocytic infiltration of the entire dermis that is most severe in the prepuce, ears, eyelids and facial skin   (MGI Ref ID J:11262)
  • scaly skin
    • by 14-15 days of age, ears, feet, tail and eyelids are scaly   (MGI Ref ID J:11262)
    • scales on base of tail may form thick circumferential rings   (MGI Ref ID J:11262)
  • skin lesions
    • apparent at 7 days of age   (MGI Ref ID J:11262)
  • respiratory system phenotype
  • pleural effusion
    • severely anemic mice may exhibit pleural effusion   (MGI Ref ID J:11262)

Foxp3sf/Y

        involves: 101/H * C3H/HeH * STOCK MR
  • mortality/aging
  • complete lethality at weaning
    • death occurs as early as P18 and most die between 19 and 22 days, although a few survive to 30 days   (MGI Ref ID J:10398)
  • growth/size/body phenotype
  • decreased body size   (MGI Ref ID J:10398)
    • decreased body weight   (MGI Ref ID J:10398)
  • postnatal growth retardation
    • from 14-18 days there is a marked retardation of growth although animals are normal in size before then   (MGI Ref ID J:10398)
  • small ears   (MGI Ref ID J:10398)
  • vision/eye phenotype
  • conjunctivitis   (MGI Ref ID J:10398)
  • narrow eye opening
    • closed eyelids in almost all 14 day or older mice due to conjunctivitis   (MGI Ref ID J:10398)
    • eyelid aperature is small   (MGI Ref ID J:10398)
  • hearing/vestibular/ear phenotype
  • small ears   (MGI Ref ID J:10398)
  • reproductive system phenotype
  • abnormal male reproductive system morphology
    • reddening and swelling of the genital papilla at P12-14   (MGI Ref ID J:10398)
    • absent scrotum   (MGI Ref ID J:10398)
    • cryptorchism
      • testes are abdominal or inguinal   (MGI Ref ID J:10398)
  • short perineum   (MGI Ref ID J:10398)
  • small gonad
    • reproductive structures are extremely underdeveloped   (MGI Ref ID J:10398)
  • immune system phenotype
  • abnormal spleen morphology   (MGI Ref ID J:10398)
    • increased spleen red pulp amount
      • hyperplasia of the red pulp   (MGI Ref ID J:10398)
    • increased spleen weight
      • about four times the normal weight   (MGI Ref ID J:10398)
    • increased spleen white pulp amount
      • hyperplasia of the white pulp   (MGI Ref ID J:10398)
    • intermingled spleen red and white pulp
      • architecture of the spleen is disrupted, with white and red pulp intermingling   (MGI Ref ID J:10398)
  • conjunctivitis   (MGI Ref ID J:10398)
  • increased leukocyte cell number   (MGI Ref ID J:10398)
  • hematopoietic system phenotype
  • abnormal erythrocyte morphology   (MGI Ref ID J:10398)
    • anemia
      • become pale and anemic 2-3 days before death   (MGI Ref ID J:10398)
    • decreased erythrocyte cell number
      • low at birth and decrease as disease progresses   (MGI Ref ID J:10398)
    • decreased hematocrit   (MGI Ref ID J:10398)
    • decreased hemoglobin content
      • older animals exhibit hypochromic red blood cells   (MGI Ref ID J:10398)
  • abnormal reticulocyte morphology
    • increased reticulocyte count   (MGI Ref ID J:10398)
  • abnormal spleen morphology   (MGI Ref ID J:10398)
    • increased spleen red pulp amount
      • hyperplasia of the red pulp   (MGI Ref ID J:10398)
    • increased spleen weight
      • about four times the normal weight   (MGI Ref ID J:10398)
    • increased spleen white pulp amount
      • hyperplasia of the white pulp   (MGI Ref ID J:10398)
    • intermingled spleen red and white pulp
      • architecture of the spleen is disrupted, with white and red pulp intermingling   (MGI Ref ID J:10398)
  • decreased megakaryocyte cell number
    • depletion of the number of megakaryocytes with age in the bone marrow   (MGI Ref ID J:10398)
    • megakaryocytes are reduced at 13 days of age, more reduced at 17 days of age, and almost absent from the spleen at 20 days of age   (MGI Ref ID J:10398)
  • decreased platelet cell number
    • low at birth and decrease as disease progresses   (MGI Ref ID J:10398)
  • extramedullary hematopoiesis
    • persistence of hematopoiesis in the liver   (MGI Ref ID J:10398)
  • increased leukocyte cell number   (MGI Ref ID J:10398)
  • liver/biliary system phenotype
  • abnormal liver morphology
    • liver is dotted with yellow foci in some animals, suggesting infection   (MGI Ref ID J:10398)
    • enlarged liver   (MGI Ref ID J:10398)
    • pale liver   (MGI Ref ID J:10398)
  • renal/urinary system phenotype
  • pale kidney   (MGI Ref ID J:10398)
  • behavior/neurological phenotype
  • lethargy
    • become lethargic the day before death   (MGI Ref ID J:10398)
  • cardiovascular system phenotype
  • gastrointestinal hemorrhage   (MGI Ref ID J:10398)
  • digestive/alimentary phenotype
  • diarrhea
    • some mice have diarrhea from the age of 14-15 days   (MGI Ref ID J:10398)
  • gastrointestinal hemorrhage   (MGI Ref ID J:10398)
  • melena
    • blood in feces is seen 2-3 days before death   (MGI Ref ID J:10398)
  • short perineum   (MGI Ref ID J:10398)
  • respiratory system phenotype
  • abnormal breathing pattern   (MGI Ref ID J:10398)
  • endocrine/exocrine gland phenotype
  • cryptorchism
    • testes are abdominal or inguinal   (MGI Ref ID J:10398)
  • craniofacial phenotype
  • small ears   (MGI Ref ID J:10398)
  • integument phenotype
  • scaly skin
    • first visible on the underside of the tail and later on other body parts   (MGI Ref ID J:10398)
  • tight skin   (MGI Ref ID J:10398)

Foxp3sf/Y

        Background Not Specified
  • immune system phenotype
  • liver inflammation
    • tissue displays extensive mononuclear cell infiltration   (MGI Ref ID J:138808)
  • lung inflammation
    • tissue displays extensive mononuclear cell infiltration   (MGI Ref ID J:138808)
    • tissue has dense predominantly peribronchovascular infiltrates, composed of CD4+ and Cd8+ T cells   (MGI Ref ID J:138808)
  • liver/biliary system phenotype
  • liver inflammation
    • tissue displays extensive mononuclear cell infiltration   (MGI Ref ID J:138808)
  • respiratory system phenotype
  • lung inflammation
    • tissue displays extensive mononuclear cell infiltration   (MGI Ref ID J:138808)
    • tissue has dense predominantly peribronchovascular infiltrates, composed of CD4+ and Cd8+ T cells   (MGI Ref ID J:138808)
View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

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

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Lymphoid Tissue Defects

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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, Separated PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Bennett CL; Brunkow ME; Ramsdell F; O'Briant KC; Zhu Q; Fuleihan RL; Shigeoka AO; Ochs HD; Chance PF. 2001. A rare polyadenylation signal mutation of the FOXP3 gene (AAUAAA-->AAUGAA) leads to the IPEX syndrome. Immunogenetics 53(6):435-9. [PubMed: 11685453]  [MGI Ref ID J:72435]

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]

Blair PJ; Carpenter DA; Godfrey VL; Russell LB; Wilkinson JE; Rinchik EM. 1994. The mouse scurfy (sf) mutation is tightly linked to Gata1 and Tfe3 on the proximal X chromosome. Mamm Genome 5(10):652-4. [PubMed: 7849405]  [MGI Ref ID J:21019]

Bultman S; Magnuson T. 2000. Molecular and genetic analysis of the mouse homolog of the Drosophila suppressor of position-effect variegation 3-9 gene Mamm Genome 11(4):251-4. [PubMed: 10754099]  [MGI Ref ID J:61103]

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]

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]

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; 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]

Means GD; Toy DY; Baum PR; Derry JM. 2000. A transcript map of a 2-Mb BAC contig in the proximal portion of the mouse X chromosome and regional mapping of the scurfy mutation. Genomics 65(3):213-23. [PubMed: 10857745]  [MGI Ref ID J:62168]

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]

Foxp3sf related

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]

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]

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]

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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]

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Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           FGB27

Colony Maintenance

Mating SystemHeterozygote x Inbred         (Female x Male)   01-MAR-06
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $199.90FemaleHeterozygous for Foxp3sf  
Price per Pair (US dollars $)Pair Genotype
$220.95Heterozygous for Foxp3sf x C57BL/6J (000664)  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $259.90FemaleHeterozygous for Foxp3sf  
Price per Pair (US dollars $)Pair Genotype
$287.30Heterozygous for Foxp3sf x C57BL/6J (000664)  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

General Supply Notes

  • View the complete collection of spontaneous mutants in the Mouse Mutant Resource.

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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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.
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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

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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.

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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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