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).
Brian Soper, The Jackson Laboratory
Genetic Background | Generation |
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|
Allele Type | Gene Symbol | Gene Name |
---|---|---|
Spontaneous | H2-K | histocompatibility 2, K region |
Allele Type |
---|
Transgenic (Inserted expressed sequence, Humanized sequence) |
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).
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.
Expressed Gene | GUSB, glucuronidase beta, human |
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Site of Expression |
Allele Name | b haplotype mutation 1 |
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Allele Type | Spontaneous |
Allele Synonym(s) | bm1; H(z1); H-2ba; H-2bm1; Kbm1 |
Gene Symbol and Name | H2-K, histocompatibility 2, K region |
Gene Synonym(s) | |
Strain of Origin | C57BL/6By |
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). |
Allele Name | transgene insertion 4, William S Sly |
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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 maintaining a live colony, these mice can be bred as hemizygotes. The Donating Investigator maintained the colony by breeding mice homozygous for the transgene.
When using the B6.Cg-H2-Kbm1 Tg(GUSB)4Sly/SndsJ mouse strain in a publication, please cite the originating article(s) and include JAX stock #006558 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
---|---|---|
Homozygous for H2-K<bm1> and Hemizygous for Tg(GUSB)4Sly |
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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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.
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