Strain Name:

B6.Cg-H2-Kbm1 Tg(GUSB)4Sly/SndsJ

Stock Number:

006558

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Availability:

Cryopreserved - Ready for recovery

Mice that are homozygous for this transgene and the Gusbmps allele have approximately 20-fold higher beta-glucuronidase enzyme activity than wildtype controls. These transgenic mice do not carry the Gusbmps allele. Mice that are hemizygous for this transgene are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. This mutant mouse strain may be useful in studies of lysomal storage diseases and mucopolysaccharidosis VII (Sly syndrome).

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.Cg-H2bm1 Tg(GUSB)4Sly/SndsJ    (Changed: 24-APR-09 )
Type Chemically Induced Mutation; Congenic; Major Histocompatibility Congenic; Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
GenerationF15pN1
Generation Definitions
 
Donating Investigator Brian Soper,   The Jackson Laboratory

Description
Mice that are homozygous for this transgene and the Gusbmps allele have approximately 20-fold higher beta-glucuronidase enzyme activity than wildtype controls. Distribution of human enzyme activity throughout various tissues mimics the endogenous mouse enzyme activity pattern. Of note, mice homozygous for both the transgene and the Gusbmps allele do not exhibit an accumulation of undegraded glycosaminoglycans. These transgenic mice do not carry the Gusbmps allele. Mice that are hemizygous for this transgene are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. This mutant mouse strain may be useful in studies of lysomal storage diseases and mucopolysaccharidosis VII (Sly syndrome).

Development
The entire human beta-glucuronidase gene with 1.6kb of 5'-flanking sequence and 3.8kb of 3'-flanking sequence was microinjected into C57BL/6J zygotes. Male mice from founder line 4 that were hemizygous for the transgenic insert were crossed with female mice homozygous for the Gusbmps allele. Mice homozygous for both the transgene and the Gusbmps allele were produced by intercross. These mice were maintained by crossing to B6.C-H2bm1/ByJ (Stock No. 001060) and no longer carry the Gusbmps allele.

The Donating Investigator maintained the colony by breeding mice homozygous for the transgene.

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   H2-Kbm1 allele
000368   B6.C-H2-Kbm1/By
006559   B6.C-H2-Kbm1/ByBir-Gusbmps/BrkJ
000256   B6.C-H2-Kbm1/ByBir-Gusbmps/J
001060   B6.C-H2-Kbm1/ByJ
002432   B6J x B6.C-H2-Kbm1/ByJ-Cdh23v-J/J
View Strains carrying   H2-Kbm1     (5 strains)

Strains carrying   Tg(GUSB)4Sly allele
001880   STOCK Gusbmps Tg(GUSB)4Sly/BirJ
View Strains carrying   Tg(GUSB)4Sly     (1 strain)

View Strains carrying other alleles of H2-K     (13 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on transgenic expression of an ortholog of a human gene that is associated with this disease. Phenotypic similarity to the human disease has not been tested.
Mucopolysaccharidosis, Type VII; MPS7   (GUSB)
View Research Applications

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

Metabolism Research

Research Tools
Metabolism Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol H2-Kbm1
Allele Name b haplotype mutation 1
Allele Type Spontaneous
Common Name(s) H(z1); H-2ba; H-2bm1; Kbm1; bm1;
Strain of OriginBALB/cBy
Gene Symbol and Name H2-K, histocompatibility 2, K region
Chromosome 17
General Note Genbank ID for this allele: X56624
Molecular Note The bm1 mutation contains 7 nucleotide differences resulting in amino acid substitutions at codon 152 (glutamate to alanine), codon 155 (arginine to tyrosine) and codon 156 (leucine to tyrosine). [MGI Ref ID J:109263] [MGI Ref ID J:109268] [MGI Ref ID J:109270] [MGI Ref ID J:164061] [MGI Ref ID J:35731]
 
Allele Symbol Tg(GUSB)4Sly
Allele Name transgene insertion 4, William S Sly
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) TgGUSB;
Strain of OriginC57BL/6J
Expressed Gene GUSB, glucuronidase, beta, human
Promoter GUSB, glucuronidase, beta, human
Molecular Note The entire human beta-glucuronidase gene with 1.6kb of 5'-flanking sequence and 3.8kb of 3'-flanking sequence was microinjected into C57BL/6J zygotes. Founder line 4 was subsequently propagated. Mice that are homozygous for this transgene and the Gusbmps allele have approximately 20-fold higher beta-glucuronidase enzyme activity than wildtype controls. Distribution of human enzyme activity throughout various tissues mimics the endogenous mouse enzyme activity pattern. [MGI Ref ID J:21256]
 

Genotyping

Genotyping Information

Genotyping Protocols

Tg(GUSB)4Sly, QPCR
Tg(GUSB)4Sly, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Kyle JW; Birkenmeier EH; Gwynn B; Vogler C; Hoppe PC; Hoffmann JW; Sly WS. 1990. Correction of murine mucopolysaccharidosis VII by a human beta-glucuronidase transgene. Proc Natl Acad Sci U S A 87(10):3914-8. [PubMed: 2111021]  [MGI Ref ID J:21256]

Additional References

H2-Kbm1 related

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Spielman J; Lee RK; Podack ER. 1998. Perforin/Fas-ligand double deficiency is associated with macrophage expansion and severe pancreatitis. J Immunol 161(12):7063-70. [PubMed: 9862744]  [MGI Ref ID J:112121]

Tabeta K; Hoebe K; Janssen EM; Xia Y; Beutler B. 2013. Respond to "No antigen-presentation defect in Unc93b1(3d/3d) (3d) mice". Nat Immunol 14(11):1102-3. [PubMed: 24145779]  [MGI Ref ID J:208726]

Wang H; Wu X; Wang Y; Oldenborg PA; Yang YG. 2010. CD47 is required for suppression of allograft rejection by donor-specific transfusion. J Immunol 184(7):3401-7. [PubMed: 20208011]  [MGI Ref ID J:160062]

Wang L; Jameson SC; Hogquist KA. 2009. Epidermal Langerhans cells are not required for UV-induced immunosuppression. J Immunol 183(9):5548-53. [PubMed: 19843938]  [MGI Ref ID J:156799]

Zhan Y; Bourges D; Dromey JA; Harrison LC; Lew AM. 2007. The origin of thymic CD4+CD25+ regulatory T cells and their co-stimulatory requirements are determined after elimination of recirculating peripheral CD4+ cells. Int Immunol 19(4):455-63. [PubMed: 17314081]  [MGI Ref ID J:119986]

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, these mice can be bred as hemizygotes. The Donating Investigator maintained the colony by breeding mice homozygous for the transgene.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Cryopreserved

Cryopreserved Mice - Ready for Recovery

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

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

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* $3283.00
Animals Provided

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

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.
 

Payment Terms and Conditions

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


See Terms of Use tab for General Terms and Conditions


The Jackson Laboratory's Genotype Promise

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

Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

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

No Warranty

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

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

No Liability

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

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

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

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


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