Strain Name:

B6.129S4-Cd247tm1Lov/J

Stock Number:

002704

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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

Former Names B6.129S4-Cd3ztm1Lov/J    (Changed: 21-MAR-06 )
Type Congenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6
Donor Strain 129S4 via J1 ES cell line
 
Donating InvestigatorDr. Paul Love,   National Institutes of Health

Appearance
black
Related Genotype: a/a

Description
Mice homozygous for the Cd3ztm1Lov targeted mutation are viable and fertile. Homozygous mutant mice exhibit impaired T-cell development and function.

Development
Neither the zeta nor the eta alternative splicing form is expressed in homozygous mice. The targeting construct cause a deletion of exon 2 and a disruption of exon 4 by insertion of neo.

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls

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.
Immunodeficiency Due to Defect in Cd3-Zeta   (CD247)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Cd247tm1Lov/Cd247tm1Lov

        B6.129S4-Cd247tm1Lov/J
  • nervous system phenotype
  • abnormal lateral geniculate nucleus morphology
    • area of the ipsilateral projection is increased compared to controls   (MGI Ref ID J:66246)
  • abnormal long term depression
    • 900 pulses at 1 Hz enhances baseline LTD, but LTD is unchanged in wild-type   (MGI Ref ID J:66246)
    • reduced long term depression
      • 900 pulses at 0.5 Hz does not induce significant LTD in contrast to wild-type response   (MGI Ref ID J:66246)
  • enhanced long term potentiation
    • LTP in response to tetanus is enhanced by approximately 248% as compared to wild type   (MGI Ref ID J:66246)
  • enlarged lateral ventricles
    • 22% of P13 mice and 20% of adult mice have enlarged lateral ventricles   (MGI Ref ID J:66246)

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

Cd247tm1Lov/Cd247tm1Lov

        involves: 129S4/SvJae
  • immune system phenotype
  • abnormal T-helper 1 cell morphology
    • Th1 memory cell phenotype   (MGI Ref ID J:14170)
  • abnormal T-helper 1 physiology
    • can be stimulated to produce large quantities of interferon gamma   (MGI Ref ID J:46914)
    • can be induced to produce Il2   (MGI Ref ID J:46914)
  • abnormal single-positive T cell number
    • reduced in number but significant numbers of single positive T-cells expressing low levels of CD5 and barely detectable levels of CD3 epsilon and TCR beta are found in lymph nodes present never the less   (MGI Ref ID J:14170)
    • deficient in both single positive thymocyte types   (MGI Ref ID J:14170)
    • decreased CD4-positive T cell number
      • about 1% normal levels in thymus   (MGI Ref ID J:14170)
  • absent gamma-delta intraepithelial T cell
    • TCR gamma-delta T cells are virtually absent while alpha-beta lineages are readily detectable among intestinal epithelial lymphocytes   (MGI Ref ID J:46914)
  • decreased double-positive T cell number
    • decreased number of double positive thymocytes   (MGI Ref ID J:14170)
  • decreased thymocyte number
    • average number of thymocytes about 14% of control numbers   (MGI Ref ID J:14170)
  • nervous system phenotype
  • abnormal CNS synaptic transmission   (MGI Ref ID J:66246)
    • abnormal excitatory postsynaptic potential
      • increased slope of the field excitatory post synaptic potential   (MGI Ref ID J:66246)
    • absent long term depression
      • not detectable   (MGI Ref ID J:66246)
    • enhanced long term potentiation
      • in CA1 of the hippocampus after titanic stimulation   (MGI Ref ID J:66246)
  • abnormal brain morphology   (MGI Ref ID J:66246)
    • abnormal lateral geniculate nucleus morphology
      • the area of ipsilateral projections to the dorsal lateral geniculate nucleus is enlarged   (MGI Ref ID J:66246)
      • ectopic projections are sometimes also found in mediodorsal areas   (MGI Ref ID J:66246)
    • abnormal superior colliculus morphology
      • retinal projections to the ipsilateral superior colliculus occasionally found   (MGI Ref ID J:66246)
  • hematopoietic system phenotype
  • abnormal T-helper 1 cell morphology
    • Th1 memory cell phenotype   (MGI Ref ID J:14170)
  • abnormal T-helper 1 physiology
    • can be stimulated to produce large quantities of interferon gamma   (MGI Ref ID J:46914)
    • can be induced to produce Il2   (MGI Ref ID J:46914)
  • abnormal single-positive T cell number
    • reduced in number but significant numbers of single positive T-cells expressing low levels of CD5 and barely detectable levels of CD3 epsilon and TCR beta are found in lymph nodes present never the less   (MGI Ref ID J:14170)
    • deficient in both single positive thymocyte types   (MGI Ref ID J:14170)
    • decreased CD4-positive T cell number
      • about 1% normal levels in thymus   (MGI Ref ID J:14170)
  • absent gamma-delta intraepithelial T cell
    • TCR gamma-delta T cells are virtually absent while alpha-beta lineages are readily detectable among intestinal epithelial lymphocytes   (MGI Ref ID J:46914)
  • decreased double-positive T cell number
    • decreased number of double positive thymocytes   (MGI Ref ID J:14170)
  • decreased thymocyte number
    • average number of thymocytes about 14% of control numbers   (MGI Ref ID J:14170)
View Research Applications

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

Cd247tm1Lov related

Immunology, Inflammation and Autoimmunity Research
CD Antigens, Antigen Receptors, and Histocompatibility Markers

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Cd247tm1Lov
Allele Name targeted mutation 1, Paul Love
Allele Type Targeted (knock-out)
Common Name(s) CD3zeta-KO; zeta -; zeta-KO; zeta/eta-;
Mutation Made ByDr. Paul Love,   National Institutes of Health
Strain of Origin129S4/SvJae
ES Cell Line NameJ1
ES Cell Line Strain129S4/SvJae
Gene Symbol and Name Cd247, CD247 antigen
Chromosome 1
Gene Common Name(s) 4930549J05Rik; AW552088; CD3 antigen, eta polypeptide; CD3 antigen, zeta polypeptide; CD3 zeta; CD3-ZETA; CD3-eta; CD3-zeta/eta; CD3H; CD3Q; CD3Z; CD3zeta; Cd3h; Cd3z; RIKEN cDNA 4930549J05 gene; T-cell receptor zeta chain; T3-zeta chain; T3Z; T3z; TCRZ; TCRk; TCRzeta; Tcrz; expressed sequence AW552088;
Molecular Note Deletion of a genomic fragment containing exon 2 and an insertion of a neomycin gene into exon 4. Northern blot analysis demonstrated that small amounts of a mutant transcript could be detected in thymocyte and lymph node RNA derived from homozygous mice. Metabolic labeling and immunoprecipitation studies also confirmed that the protein was not detectable in thymocytes from homozygous mice. [MGI Ref ID J:72520]

Genotyping

Genotyping Information

Genotyping Protocols

Cd247tm1Lov, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Love PE; Shores EW; Johnson MD; Tremblay ML; Lee EJ; Grinberg A; Huang SP; Singer A; Westphal H. 1993. T cell development in mice that lack the zeta chain of the T cell antigen receptor complex. Science 261(5123):918-21. [PubMed: 7688481]  [MGI Ref ID J:14170]

Additional References

Cd247tm1Lov related

Barco A; Patterson S; Alarcon JM; Gromova P; Mata-Roig M; Morozov A; Kandel ER. 2005. Gene expression profiling of facilitated L-LTP in VP16-CREB mice reveals that BDNF is critical for the maintenance of LTP and its synaptic capture. Neuron 48(1):123-37. [PubMed: 16202713]  [MGI Ref ID J:105355]

Chiesa S; Mingueneau M; Fuseri N; Malissen B; Raulet DH; Malissen M; Vivier E; Tomasello E. 2006. Multiplicity and plasticity of natural killer cell signaling pathways. Blood 107(6):2364-72. [PubMed: 16291591]  [MGI Ref ID J:125723]

D'Oro U; Munitic I; Chacko G; Karpova T; McNally J; Ashwell JD. 2002. Regulation of constitutive TCR internalization by the zeta-chain. J Immunol 169(11):6269-78. [PubMed: 12444133]  [MGI Ref ID J:112127]

Deng GM; Beltran J; Chen C; Terhorst C; Tsokos GC. 2013. T cell CD3zeta deficiency enables multiorgan tissue inflammation. J Immunol 191(7):3563-7. [PubMed: 23980209]  [MGI Ref ID J:205963]

Guy CS; Vignali KM; Temirov J; Bettini ML; Overacre AE; Smeltzer M; Zhang H; Huppa JB; Tsai YH; Lobry C; Xie J; Dempsey PJ; Crawford HC; Aifantis I; Davis MM; Vignali DA. 2013. Distinct TCR signaling pathways drive proliferation and cytokine production in T cells. Nat Immunol 14(3):262-70. [PubMed: 23377202]  [MGI Ref ID J:193617]

Hayes SM; Li L; Love PE. 2005. TCR signal strength influences alphabeta/gammadelta lineage fate. Immunity 22(5):583-93. [PubMed: 15894276]  [MGI Ref ID J:99104]

Holst J; Wang H; Eder KD; Workman CJ; Boyd KL; Baquet Z; Singh H; Forbes K; Chruscinski A; Smeyne R; van Oers NS; Utz PJ; Vignali DA. 2008. Scalable signaling mediated by T cell antigen receptor-CD3 ITAMs ensures effective negative selection and prevents autoimmunity. Nat Immunol 9(6):658-66. [PubMed: 18469818]  [MGI Ref ID J:136207]

Huh GS; Boulanger LM; Du H; Riquelme PA; Brotz TM; Shatz CJ. 2000. Functional requirement for class I MHC in CNS development and plasticity Science 290(5499):2155-9. [PubMed: 11118151]  [MGI Ref ID J:66246]

Hwang S; Song KD; Lesourne R; Lee J; Pinkhasov J; Li L; El-Khoury D; Love PE. 2012. Reduced TCR signaling potential impairs negative selection but does not result in autoimmune disease. J Exp Med 209(10):1781-95. [PubMed: 22945921]  [MGI Ref ID J:192915]

Kumar R; Ferez M; Swamy M; Arechaga I; Rejas MT; Valpuesta JM; Schamel WW; Alarcon B; van Santen HM. 2011. Increased Sensitivity of Antigen-Experienced T Cells through the Enrichment of Oligomeric T Cell Receptor Complexes. Immunity 35(3):375-87. [PubMed: 21903423]  [MGI Ref ID J:176665]

Love PE; Tremblay ML; Westphal H. 1992. Targeting of the T-cell receptor zeta-chain gene in embryonic stem cells: strategies for generating multiple mutations in a single gene. Proc Natl Acad Sci U S A 89(20):9929-33. [PubMed: 1409721]  [MGI Ref ID J:72520]

She J; Ruzek MC; Velupillai P; de Aos I; Wang B; Harn DA; Sancho J; Biron CA; Terhorst C. 1999. Generation of antigen-specific cytotoxic T lymphocytes and regulation of cytokine production takes place in the absence of CD3zeta. Int Immunol 11(5):845-57. [PubMed: 10330289]  [MGI Ref ID J:55404]

Shores E; Flamand V; Tran T; Grinberg A; Kinet JP; Love PE. 1997. Fc epsilonRI gamma can support T cell development and function in mice lacking endogenous TCR zeta-chain. J Immunol 159(1):222-30. [PubMed: 9200458]  [MGI Ref ID J:41075]

Shores EW; Huang K; Tran T; Lee E; Grinberg A; Love PE. 1994. Role of TCR zeta chain in T cell development and selection. Science 266(5187):1047-50. [PubMed: 7526464]  [MGI Ref ID J:79623]

Shores EW; Ono M; Kawabe T; Sommers CL; Tran T; Lui K; Udey MC; Ravetch J; Love PE. 1998. T cell development in mice lacking all T cell receptor zeta family members (Zeta, eta, and FcepsilonRIgamma). J Exp Med 187(7):1093-101. [PubMed: 9529325]  [MGI Ref ID J:46914]

Shores EW; Tran T; Grinberg A; Sommers CL; Shen H; Love PE. 1997. Role of the multiple T cell receptor (TCR)-zeta chain signaling motifs in selection of the T cell repertoire. J Exp Med 185(5):893-900. [PubMed: 9120395]  [MGI Ref ID J:147410]

Tomasello E; Desmoulins PO; Chemin K; Guia S; Cremer H; Ortaldo J; Love P; Kaiserlian D; Vivier E. 2000. Combined natural killer cell and dendritic cell functional deficiency in KARAP/DAP12 loss-of-function mutant mice. Immunity 13(3):355-64. [PubMed: 11021533]  [MGI Ref ID J:64764]

Vaknin I; Blinder L; Wang L; Gazit R; Shapira E; Genina O; Pines M; Pikarsky E; Baniyash M. 2008. A common pathway mediated through Toll-like receptors leads to T- and natural killer-cell immunosuppression. Blood 111(3):1437-47. [PubMed: 17991807]  [MGI Ref ID J:130783]

Xu HP; Chen H; Ding Q; Xie ZH; Chen L; Diao L; Wang P; Gan L; Crair MC; Tian N. 2010. The immune protein CD3zeta is required for normal development of neural circuits in the retina. Neuron 65(4):503-15. [PubMed: 20188655]  [MGI Ref ID J:167764]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

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

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* $2450.00
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 $1600.00

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 11 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* $3185.00
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 $2080.00

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 11 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.

Control Information

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

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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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