Strain Name:

C3H/HeOuJ-Gusbmps-2J/BrkJ

Stock Number:

003525

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Availability:

Cryopreserved - Ready for recovery

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 C3H/HeOuJ-Gusbmps-2J    (Changed: 13-APR-07 )
C3H/HeOuJ-Gusmps-2J    (Changed: 15-DEC-04 )
Type Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse

Appearance
black
Related Genotype: a/a

Description
Mice homozygous for the Gusmps-2J allele exhibit a phenotype similar to Gusmps homozygotes including skeletal deformities, lysosomal storage disease and elevated levels of the lysosomal enzymes alpha-galactosidase and beta-hexosaminidase (Gwynn et al., 1998). Like the Gusmps heterozygote, Gusmps-2J heterozygotes have a 26-85% reduction in beta-glucuronidase activity. depending on tissue type (Gwynn et al., 1998, Birkenmeier et al., 1989). Homozygotes of both alleles have beta-glucuronidase activity levels at 1% of the control level. Unlike the Gusmps homozygote, Gusmps-2J homozygotes live longer, are fertile and can raise litters to weaning age (Gwynn et al., 1998). In addition to a difference in the nature of the mutations between these two alleles, it is also likely that the phenotypic differences are the result of strain background. C3H/HeOuJ mice carry a different set of alleles at the Gus complex than C57BL/6J mice. Beta-glucuronidase activity in C3H/HeOuJ mice is 10-38% that of C57BL/6J mice (Gwynn et al., 1998). The Gus complex alleles determine the rate of enzyme synthesis and may modulate the expression of the beta-glucuronidase deficiency. This strain is a model for the human lysomal storage disease, mucopolysaccharidosis type VII.

Development
The Gusmps-2J mutation arose spontaneously in the C3H/HeOuJ (Stock No. 000635) production colony at The Jackson Laboratory (Gwynn et al., 1998). The founder male was identified by a blunter face and shorter, thickened limbs and tail. This strain was maintained by sibling inbreeding and in 2007 sperm were cryopreserved from homozygous males at generation F46.

Control Information

  Control
   000635 C3H/HeOuJ
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Gusbmps-2J allele
006557   B6.C3-Gusbmps-2J/BrkJ
View Strains carrying   Gusbmps-2J     (1 strain)

View Strains carrying other alleles of Gusb     (19 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).
Mucopolysaccharidosis, Type VII; MPS7
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Gusbmps-2J/Gusbmps-2J

        C3H/HeOuJ-Gusbmps-2J/BrkJ
  • mortality/aging
  • premature death   (MGI Ref ID J:72372)
  • cellular phenotype
  • abnormal lysosome morphology
    • lysosomal storage disorder   (MGI Ref ID J:50358)
    • lysosomal storage disorder   (MGI Ref ID J:72372)
  • craniofacial phenotype
  • shortened head   (MGI Ref ID J:50358)
  • growth/size/body phenotype
  • decreased body size   (MGI Ref ID J:50358)
  • postnatal growth retardation   (MGI Ref ID J:50358)
  • limbs/digits/tail phenotype
  • short limbs   (MGI Ref ID J:50358)
  • short tail   (MGI Ref ID J:50358)
  • thick tail   (MGI Ref ID J:50358)
View Research Applications

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

Gusbmps-2J related

Developmental Biology Research
Growth Defects
      Growth Defects (homozygous)
Skeletal Defects

Metabolism Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Gusbmps-2J
Allele Name beta glucuronidase, mucopolysaccharidosis VII 2 Jackson
Allele Type Spontaneous
Common Name(s) gusmps-2J;
Mutation Made By Brian Soper,   The Jackson Laboratory
Strain of OriginC3H/HeOuJ
Gene Symbol and Name Gusb, glucuronidase, beta
Chromosome 5
Gene Common Name(s) AI747421; Ac2-223; BG; Gur; Gus; Gus-r; Gus-s; Gus-t; Gus-u; Gut; MPS7; adipose storage deficiency; asd; beta-glucuronidase regulator; beta-glucuronidase structural; beta-glucuronidase systemic regulator; beta-glucuronidase temporal; expressed sequence AI747421; g;
Molecular Note Southern blot analysis showed that this allele is the result of a 5.4kb intracisternal A particle (IAP) transposon insertion near the 3' end of intron 8. Homozygous mice have less than 1% of the normal levels of beta glucuronidase activity. [MGI Ref ID J:50358]

Genotyping

Genotyping Information

Genotyping Protocols

Gusbmps-2J, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Gwynn B; Lueders K; Sands MS; Birkenmeier EH. 1998. Intracisternal A-particle element transposition into the murine beta-glucuronidase gene correlates with loss of enzyme activity: a new model for beta-glucuronidase deficiency in the C3H mouse. Mol Cell Biol 18(11):6474-81. [PubMed: 9774663]  [MGI Ref ID J:50358]

Additional References

Birkenmeier EH; Davisson MT; Beamer WG; Ganschow RE; Vogler CA; Gwynn B; Lyford KA; Maltais LM; Wawrzyniak CJ. 1989. Murine mucopolysaccharidosis type VII. Characterization of a mouse with beta-glucuronidase deficiency. J Clin Invest 83(4):1258-6. [PubMed: 2495302]  [MGI Ref ID J:9705]

Sands MS; Birkenmeier EH. 1993. A single-base-pair deletion in the beta-glucuronidase gene accounts for the phenotype of murine mucopolysaccharidosis type VII. Proc Natl Acad Sci U S A 90(14):6567-71. [PubMed: 8101990]  [MGI Ref ID J:13207]

Sands MS; Vogler C; Kyle JW; Grubb JH; Levy B; Galvin N; Sly WS; Birkenmeier EH. 1994. Enzyme replacement therapy for murine mucopolysaccharidosis type VII. J Clin Invest 93(6):2324-31. [PubMed: 8200966]  [MGI Ref ID J:19122]

Vogler C; Levy B; Galvin N; Sands MS; Birkenmeier EH; Sly WS; Barker J. 2001. A novel model of murine mucopolysaccharidosis type VII due to an intracisternal a particle element transposition into the beta-glucuronidase gene: clinical and pathologic findings. Pediatr Res 49(3):342-8. [PubMed: 11228259]  [MGI Ref ID J:72372]

Gusbmps-2J related

Vogler C; Levy B; Galvin N; Sands MS; Birkenmeier EH; Sly WS; Barker J. 2001. A novel model of murine mucopolysaccharidosis type VII due to an intracisternal a particle element transposition into the beta-glucuronidase gene: clinical and pathologic findings. Pediatr Res 49(3):342-8. [PubMed: 11228259]  [MGI Ref ID J:72372]

Zhou D; Cantu C rd; Sagiv Y; Schrantz N; Kulkarni AB; Qi X; Mahuran DJ; Morales CR; Grabowski GA; Benlagha K; Savage P; Bendelac A; Teyton L. 2004. Editing of CD1d-bound lipid antigens by endosomal lipid transfer proteins. Science 303(5657):523-7. [PubMed: 14684827]  [MGI Ref ID J:90443]

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* $2085.00
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 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* $2710.50
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 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.

General Supply Notes

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

Control Information

  Control
   000635 C3H/HeOuJ
 
  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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