These mice carry a spontaneous mutation at the Gusb locus, that by itself causes a deficiency of beta-glucuronidase, and a transgene carrying the human beta-glucuronidase gene, that corrects the beta-glucuronidase deficiency. These mice are useful in studies relating to mucopolysaccharidosis VII (Sly syndrome) in humans.
Read More +Genetic Background | Generation |
---|---|
|
Allele Type | Gene Symbol | Gene Name |
---|---|---|
Spontaneous | Gusb | glucuronidase, beta |
Allele Type |
---|
Transgenic (Inserted expressed sequence, Humanized sequence) |
These mice carry an allele that confers a deficiency of beta-glucuronidase (beta-D-glucuronoside glucuronosohydrolase) and a transgene carrying the human beta-glucuronidase gene. Whereas the Gusbmps allele produces virtually no murine beta-glucuronidase, these mice with the human beta-glucuronidase gene express high levels of human beta-glucuronidase activity in all tissues examined and are phenotypically normal. . Biochemically, both the intralysosomal storage of glycosaminoglycans and the secondary elevation of other acid hydrolases are corrected.
Expressed Gene | GUSB, glucuronidase beta, human |
---|---|
Site of Expression |
Allele Name | beta glucuronidase, mucopolysaccharidosis VII |
---|---|
Allele Type | Spontaneous |
Allele Synonym(s) | asd; gusmps; Gus-b; MPS VII |
Gene Symbol and Name | Gusb, glucuronidase, beta |
Gene Synonym(s) | |
Strain of Origin | B6.C-H2-Kbm1/By |
Chromosome | 5 |
Molecular Note | A 1-bp deletion creates a frameshift mutation within exon 10, which introduces a premature stop codon at codon 497. |
Mutations Made By | Brian Soper, The Jackson Laboratory |
Allele Name | transgene insertion 4, William S Sly |
---|---|
Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | TgGUSB |
Gene Symbol and Name | Tg(GUSB)4Sly, transgene insertion 4, William S Sly |
Gene Synonym(s) | |
Promoter | GUSB, glucuronidase beta, human |
Expressed Gene | GUSB, glucuronidase beta, human |
Strain of Origin | C57BL/6J |
Chromosome | UN |
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. |
When using the MPS VII GUSB mouse strain in a publication, please cite the originating article(s) and include JAX stock #001880 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
---|---|---|
Homozygous for Tg(GUSB)4Sly, 1 pair minimum |
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.
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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.
What information were you hoping to find through your search?
How easy was it to find what you were looking for?
We may wish to follow up with you. Enter your email if you are happy for us to connect and reachout to you with more questions.
Please Enter a Valid Email Address
Thank you for sharing your feedback! We are working on improving the JAX Mice search. Come back soon for exciting changes.