Strain Name: |
B6.C-H2bm1/ByBir-Gusbmps/J |
|---|---|
Stock Number: |
000256 |
Availability: | Repository-Cryopreserved |
General Terms and Conditions |
| Former Name |
B6.C-H2bm1/ByBir-Gusmps/J (Changed: 15-DEC-04
) |
| Genes & Alleles | Gusb; Gusbmps; H2-K; H2-Kbm1; H2; ; |
Product Information
Strain Details
Type JAX® GEMM® Strain - Congenic Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Major Histocompatibility Congenic Type JAX® GEMM® Strain - Mutant Strain Species laboratory mouse Appearance
black
Related Genotype: a/aStrain Description
Mice homozygous for the Gusbmps allele exhibit skeletal dysplasia as well as cognitive, hearing and visual deficits. Lifespan of the homozygotes is approximately six months. Homozygotes lack the lysomal enzyme, beta-glucoronidase, and, as a result, glycosaminoglycans accumulate in tissues throughout the body. Female homozygotes rarely conceive and do not lactate. This strain is a model for the human lysomal storage disease, mucopolysaccharidosis type VII.Strain Development
The Gusbmps mutation arose spontaneously on B6.C-H2bm1/By in 1976 (Birkenmeier et al., 1989).
Related Disease (OMIM) Terms |
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Gusbmps | ||
|---|---|---|---|
| Allele Name | beta glucuronidase, mucopolysaccharidosis VII | ||
| Common Name(s) | Gus-b; asd; gusmps; | ||
| Mutation Made By | Brian Soper, The Jackson Laboratory | ||
| Strain of Origin | C57BL/6By | ||
| Gene Symbol and Name | Gusb, glucuronidase, beta | ||
| Chromosome | 5 | ||
| Gene Common Name(s) | AI747421; Ac2-223; FLJ39445; 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 | A 1-bp deletion creates a frameshift mutation within exon 10, which introduces a premature stop codon at codon 497. [MGI Ref ID J:13207] | ||
| Gene Symbol and Name | H2, histocompatibility-2, MHC | ||
| Chromosome | 17 | ||
| Gene Common Name(s) | H-2; MHC-II; | ||
| Allele Symbol | H2-Kbm1 | ||
| Allele Name | b haplotype mutation 1 | ||
| Common Name(s) | H(z1); H-2ba; H-2bm1; bm1; | ||
| Gene Symbol and Name | H2-K, histocompatibility 2, K region | ||
| Chromosome | 17 | ||
Control Information
| Control | ||
|---|---|---|
| Heterozygote from the colony | ||
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying Gusbmps allele
006564 B6(C)-KitW-41J Gusbmps/BrkJ 006559 B6.C-H2bm1/ByBir-Gusbmps/BrkJ 006562 B6.CBy(Cg)-Gusbmps Gpi1a-m1J/BrkJ 006407 B6.Cg-Gusbmps/BrkJ 002086 B6.Cg-Gusbmps Tg(Gussx)1Wat/J 005053 NOD.Cg-Prkdcscid Gusbmps/SndsJ 001880 STOCK Gusbmps Tg(GUSB)4Sly/BirJ View Strains carrying Gusbmps (7 strains)
000368 B6.C-H2bm1/By 006559 B6.C-H2bm1/ByBir-Gusbmps/BrkJ 001060 B6.C-H2bm1/ByJ 006558 B6.Cg-H2bm1 Tg(GUSB)4Sly/SndsJ 002432 B6J x B6.C-H2bm1/ByJ-Cdh23v-J/J
Additional Web Information
Congenic Nomenclature
Research Applications
This mouse can be used to support research in many areas including:Gusbmps related
Developmental Biology Research
Growth Defects Growth Defects (homozygous)
Skeletal Defects
Metabolism Research
Mouse/Human Gene Homologs
mucopolysaccharidosis type VII, GUSB deficiency
Neurobiology Research
Behavioral and Learning Defects
Sensorineural Research
References
Additional ReferencesPrice and Supply Information
| Strain Name: | B6.C-H2bm1/ByBir-Gusbmps/J |
| Stock Number: | 000256 |
Price Details
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Supply Details
| Standard Supply | Repository-Cryopreserved. Must Be Recovered. Please refer to the Supply Notes for further information. |
|---|---|
| Supply Notes |
Cryorecovery of Strains Needing Progeny Testing. The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two untested males and two untested females (two pairs) will be recovered, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. However, all pups recovered will be sent. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals (at least two untested pairs) to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation. Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 or 1-207-288-5845. Cryorecovery to establish a Dedicated Supply for greater quantities of mice |
| Licensing | See General Terms and Conditions below |
| Control Information | View Control Information in Strain Details. |
General Terms and Conditions
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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.Ordering and Purchasing Information
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