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Strain Name:

STOCK Gusbmps Tg(GUSB)4Sly/BirJ

Stock Number:

001880

Availability:

Repository-Cryopreserved


General Terms and Conditions

Former Name      STOCK Tg(mpsGUSB)13Bir/J    (Changed: 06-JUN-08 )
Genes & Alleles   GUSB;   Gusb;   Gusbmps;   Tg(GUSB)4Sly;


Product Information

Strain Details

Type JAX® GEMM® Strain - Mutant Stock
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Spontaneous Mutation
Type JAX® GEMM® Strain - Transgenic
Specieslaboratory mouse
Mammalian Phenotype Terms assigned by genotype

Gusbmps/Gusbmps Tg(GUSB)4Sly/Tg(GUSB)4Sly

        involves: C57BL/6By * C57BL/6J * LT/Sv * SJL/J
  • normal phenotype
  • no abnormal phenotype detected (MGI Ref ID J:21256)
    • mutant mice appear phenotypically normal; transgene expression rescues the lysosomal enzyme elevation, glycosaminoglycan storage, dwarfism, abnormal gait, limited joint mobility, skeletal deformities, and paucity of adipose tissue that are seen in Gusb homozygotes

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 OriginC57BL/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]
 
Allele Symbol Tg(GUSB)4Sly
Allele Name transgene insertion 4, William S Sly
Common Name(s) TgGUSB;
Strain of OriginC57BL/6J
Expressed Gene Gusb, glucuronidase, beta, mouse, laboratory
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]

Related Strains

View Strains carrying   Gusbmps     (7 strains)

Strains carrying   Tg(GUSB)4Sly allele
006558   B6.Cg-H2bm1 Tg(GUSB)4Sly/SndsJ
View Strains carrying   Tg(GUSB)4Sly     (1 strain)

Strains carrying other alleles of Gusb
005643   B6.129X-Gusbtm1Sly/J
005644   B6.129X-Gusbtm3Sly/J
006557   B6.C3-Gusbmps-2J/BrkJ
003525   C3H/HeOuJ-Gusbmps-2J/BrkJ
005322   C57BL/6J-Gusbmps-3J/J
View Strains carrying other alleles of Gusb     (5 strains)

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

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

Price and Supply Information

Strain Name: STOCK Gusbmps Tg(GUSB)4Sly/BirJ
Stock Number: 001880

Price Details

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Supply Details

Standard SupplyRepository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes Cryorecovery - Standard.
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 males and two females (two pairs) will be provided, 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 delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that 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. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery.

Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services: Tel: 1-800-422-6423 or 1-207-288-5845; Email: jaxservices@jax.org.

LicensingSee General Terms and Conditions below  

General Terms and Conditions

View JAX® Mice & Services Conditions of Use.

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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