Strain Name:

B6.129X1-Fzd9tm1Uta/J

Stock Number:

006199

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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; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Generation?N1F3pN1
Generation Definitions
 
Donating InvestigatorDr. Uta Francke,   Stanford University School of Medicine

Description
Mice homozygous for this targeted mutation are viable and fertile, with no gross anatomical abnormalities. Endogenous transcripts are absent in skeletal muscle and testis. Homozygotes exhibit abnormal B-cell development, reduced survival, lymphadenopathy secondary to accumulation of plasma cells, splenomegaly, and accelerated thymic atrophy. Mutant mice do not exhibit any obvious features of Williams-Beuren syndrome (WBS). These mice may be useful in studies of hematopoietic/lymphoid development and function (including B-cell and T-cell development), plasma cell homeostasis, and the Wnt/frizzled signaling pathway.

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. This is the case for this strain. It should be noted that the phenotype could vary from that originally published. We will modify the strain description if necessary as published results become available. While the original publication (Ranheim et al 2005 Blood 105:2487-94) describes homozygous mutant mice on a 129 genetic background, the donating investigator reports that no differences in behavioral/cognitive, cardiovascular, or other phenotyping are observed in the C57BL/6J-backcrossed colonies from which The Jackson Laboratory obtained the mice (2006).

Development
A targeting vector was designed to replace the complete open reading frame of the endogenous gene with a neomycin resistance gene. The creator of this strain/donating investigator reports (correcting to the original publication) that this construct was electroporated into 129X1/SvJ-derived JM-1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6 blastocysts. Chimeric mice were bred with C57BL/6 females. Mutant mice were backcrossed to C57BL/6J for more than 10 generations prior to arrival at The Jackson Laboratory.

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
- Model with phenotypic similarity to human disease where etiologies are distinct. Human genes are associated with this disease. Orthologs of these genes do not appear in the mouse genotype(s).
Williams-Beuren Syndrome; WBS
- 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.
Williams-Beuren Syndrome; WBS
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

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

Fzd9tm1Uta/Fzd9+

        involves: 129X1/SvJ
  • skeleton phenotype
  • abnormal bone ossification
    • decrease in bone rate formation   (MGI Ref ID J:169924)
  • decreased bone mineral density
  • decreased bone strength
    • vertebral bodies and femora exhibit decreased biomechanical competence   (MGI Ref ID J:169924)
  • decreased compact bone thickness
    • reduction in cortical thickness of femurs   (MGI Ref ID J:169924)
  • decreased osteoblast cell number   (MGI Ref ID J:169924)
  • decreased trabecular bone volume   (MGI Ref ID J:169924)

Fzd9tm1Uta/Fzd9tm1Uta

        involves: 129S6/SvEvTac * 129X1/SvJ
  • mortality/aging
  • premature death
    • exhibit moderately reduced lifespans   (MGI Ref ID J:98133)
  • immune system phenotype
  • abnormal lymph node morphology
    • variably distorted nodal architecture   (MGI Ref ID J:98133)
    • abnormal lymph node medullary cord morphology
      • an accumulation of plasma cells that filled and distended the medullary cords and were present in large patches in the paracortical zones   (MGI Ref ID J:98133)
    • enlarged lymph nodes
      • lymph nodes are frequently enlarged, even at 3 months of age, with more than 50% of older homozygous null mice showing enlarged lymph nodes   (MGI Ref ID J:98133)
  • abnormal spleen marginal zone morphology
    • although the white pulp is intact, there is some expansion of the marginal zones   (MGI Ref ID J:98133)
  • abnormal spleen red pulp morphology
    • expansion of the red pulp with increased extramedullary hematopoiesis and deposition of increased amounts of hemosiderin   (MGI Ref ID J:98133)
  • decreased B cell number
    • decreased total B-cell number in the bone marrow despite relatively normal numbers of mature B cells in the periphery   (MGI Ref ID J:98133)
    • decreased pre-B cell number
      • reduction in CD43+B220+ pro/pre-B-cell populations   (MGI Ref ID J:98133)
  • decreased pro-B cell number
    • reduction in CD43+B220+ pro/pre-B-cell populations   (MGI Ref ID J:98133)
  • enlarged spleen
    • spleens are approximately twice the normal size by 6 months of age   (MGI Ref ID J:98133)
  • increased leukocyte cell number
    • older homozygous null mice have a high frequency of peripheral blood leukocytosis   (MGI Ref ID J:98133)
    • increased eosinophil cell number
      • older homozygous null mice exhibit eosinophilia   (MGI Ref ID J:98133)
    • increased monocyte cell number
      • older homozygous null mice exhibit monocytosis   (MGI Ref ID J:98133)
    • increased neutrophil cell number
      • older homozygous null mice exhibit mild neutrophilia   (MGI Ref ID J:98133)
  • thymus atrophy
    • exhibit accelerated atrophy with age   (MGI Ref ID J:98133)
  • hematopoietic system phenotype
  • abnormal spleen marginal zone morphology
    • although the white pulp is intact, there is some expansion of the marginal zones   (MGI Ref ID J:98133)
  • abnormal spleen red pulp morphology
    • expansion of the red pulp with increased extramedullary hematopoiesis and deposition of increased amounts of hemosiderin   (MGI Ref ID J:98133)
  • decreased B cell number
    • decreased total B-cell number in the bone marrow despite relatively normal numbers of mature B cells in the periphery   (MGI Ref ID J:98133)
    • decreased pre-B cell number
      • reduction in CD43+B220+ pro/pre-B-cell populations   (MGI Ref ID J:98133)
  • decreased pro-B cell number
    • reduction in CD43+B220+ pro/pre-B-cell populations   (MGI Ref ID J:98133)
  • enlarged spleen
    • spleens are approximately twice the normal size by 6 months of age   (MGI Ref ID J:98133)
  • increased leukocyte cell number
    • older homozygous null mice have a high frequency of peripheral blood leukocytosis   (MGI Ref ID J:98133)
    • increased eosinophil cell number
      • older homozygous null mice exhibit eosinophilia   (MGI Ref ID J:98133)
    • increased monocyte cell number
      • older homozygous null mice exhibit monocytosis   (MGI Ref ID J:98133)
    • increased neutrophil cell number
      • older homozygous null mice exhibit mild neutrophilia   (MGI Ref ID J:98133)
  • thymus atrophy
    • exhibit accelerated atrophy with age   (MGI Ref ID J:98133)
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype
    • do not exhibit any neurological, reflex or behavioral problems or hyperacusis   (MGI Ref ID J:98133)
  • endocrine/exocrine gland phenotype
  • thymus atrophy
    • exhibit accelerated atrophy with age   (MGI Ref ID J:98133)

Fzd9tm1Uta/Fzd9tm1Uta

        involves: 129X1/SvJ
  • skeleton phenotype
  • abnormal skeleton development   (MGI Ref ID J:169924)
    • abnormal osteoblast differentiation
      • osteoblastogenesis defect   (MGI Ref ID J:169924)
  • abnormal skeleton physiology   (MGI Ref ID J:169924)
    • abnormal bone ossification
      • 45% reduction in bone formation rate   (MGI Ref ID J:169924)
      • abnormal bone mineralization
        • isolated bone marrow cells grown in culture exhibit reduced mineralization after differentiation into osteoblasts but normal osteoclastogenesis   (MGI Ref ID J:169924)
        • calvaria-derived osteoblasts grown in culture for up to 20 days exhibit poor mineralization   (MGI Ref ID J:169924)
    • abnormal osteoblast physiology
      • calvaria-derived osteoblasts grown in culture in the presence of ascorbate and beta-glycerophosphate exhibit a decrease in proliferation at day 2 of differentiation   (MGI Ref ID J:169924)
    • decreased bone strength
      • vertebral bodies and femora exhibit decreased biomechanical competence   (MGI Ref ID J:169924)
  • abnormal vertebral body morphology
    • reduction in vertebral bodies by 35% and 40% at 24 and 52 weeks of age, respectively   (MGI Ref ID J:169924)
  • decreased bone mineral density
  • decreased bone trabecula number   (MGI Ref ID J:169924)
  • decreased bone volume   (MGI Ref ID J:169924)
  • decreased compact bone thickness
    • reduction in cortical thickness of femurs   (MGI Ref ID J:169924)
  • decreased osteoblast cell number
    • 30% reduction is osteoblast number   (MGI Ref ID J:169924)
    • however, no differences in osteoclast number   (MGI Ref ID J:169924)
  • cellular phenotype
  • abnormal osteoblast differentiation
    • osteoblastogenesis defect   (MGI Ref ID J:169924)
View Research Applications

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

Developmental Biology Research
Internal/Organ Defects
      hematopoietic defects
Lymphoid Tissue Defects
      hematopoietic defects

Hematological Research
Hematopoietic Defects

Immunology, Inflammation and Autoimmunity Research
Immunodeficiency
      B cell defects
Lymphoid Tissue Defects
      hematopoietic development

Internal/Organ Research
Spleen Defects
Thymus Defects

Research Tools
Immunology, Inflammation and Autoimmunity Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Fzd9tm1Uta
Allele Name targeted mutation 1, Uta Francke
Allele Type Targeted (Null/Knockout)
Common Name(s) Fzd9-;
Mutation Made ByDr. Uta Francke,   Stanford University School of Medicine
Strain of Origin129X1/SvJ
ES Cell Line NameJM-1
ES Cell Line Strain129X1/SvJ
Gene Symbol and Name Fzd9, frizzled homolog 9 (Drosophila)
Chromosome 5
Gene Common Name(s) CD349; FZD3; frizzled 9; mfz9;
Molecular Note A neomycin resistance cassette replaced the coding exon. Northern blot analysis confirmed the absence of transcription. [MGI Ref ID J:98133]

Genotyping

Genotyping Information

Genotyping Protocols

Fzd9tm1Uta, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Fzd9tm1Uta related

Albers J; Schulze J; Beil FT; Gebauer M; Baranowsky A; Keller J; Marshall RP; Wintges K; Friedrich FW; Priemel M; Schilling AF; Rueger JM; Cornils K; Fehse B; Streichert T; Sauter G; Jakob F; Insogna KL; Pober B; Knobeloch KP; Francke U; Amling M; Schinke T. 2011. Control of bone formation by the serpentine receptor Frizzled-9. J Cell Biol 192(6):1057-72. [PubMed: 21402791]  [MGI Ref ID J:169924]

Heilmann A; Schinke T; Bindl R; Wehner T; Rapp A; Haffner-Luntzer M; Nemitz C; Liedert A; Amling M; Ignatius A. 2013. The Wnt serpentine receptor Frizzled-9 regulates new bone formation in fracture healing. PLoS One 8(12):e84232. [PubMed: 24391920]  [MGI Ref ID J:211112]

Ranheim EA; Kwan HC; Reya T; Wang YK; Weissman IL; Francke U. 2005. Frizzled 9 knock-out mice have abnormal B-cell development. Blood 105(6):2487-94. [PubMed: 15572594]  [MGI Ref ID J:98133]

Wu QL; Zierold C; Ranheim EA. 2009. Dysregulation of Frizzled 6 is a critical component of B-cell leukemogenesis in a mouse model of chronic lymphocytic leukemia. Blood 113(13):3031-9. [PubMed: 19179304]  [MGI Ref ID J:147170]

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 & HusbandryWhen maintaining a live colony, mice are bred as homozygotes.

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* $2140.00
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.

Standard Supply

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

Supply Notes

  • 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* $2782.00
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.

Standard Supply

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

Supply Notes

  • 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.

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'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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"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.

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