These transgenic mice express a human TATA box binding protein, TBP, containing a 71 polyQ repeat expansion, under the control of the mouse prion protein promoter. Hemizygotes exhibit weight loss, locomotor impairment, gliosis and neuron loss. This transgenic strain has a less severe phenotype than B6CBA(FVB)-Tg(Prnp-TBP*)105Xjl/J (Stock No. 008075). B6CBA(FVB)-Tg(Prnp-TBP*)13Xjl/J (Stock No. 008083) can be used a control for this strain. This mutant mouse strain may be useful in studies of spinocerebellar ataxia 17 (SCA17) and polyglutamine (polyQ) related neurodegeneration.
Shihua Li, Emory University School of Medicine
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence, Humanized sequence) |
These transgenic mice express human TATA box binding protein,TBP, containing a 71 polyQ repeat expansion, under the control of the mouse prion protein promoter. The 71 polyQ-expansion is detected by Western blot analysis. At 6.5 months of age, hemizygous transgenic mice are smaller than wildtype littermates and appear ungroomed. These transgenic mice have a shortened lifespan of approximately 9 months and begin to die as early as 21 weeks of age. Immunohistochemical and Western blot analysis reveals visible nuclear aggregates of polyQ tracts by 15 weeks of age. Electron microscopic examination of brain tissue shows degeneration of Purkinje cells and axons. Reactive gliosis is observed in the granular and Purkinje layers. Onset of progressive locomotor impairment is 7 weeks of age. Some mice exhibit clasping, spontaneous seizures and tremors. The donating investigator has not attempted to make the strain homozygous. This transgenic strain has a less severe phenotype than B6CBA(FVB)-Tg(Prnp-TBP*)105Xjl/J (Stock No. 008075). B6CBA(FVB)-Tg(Prnp-TBP*)13Xjl/J (Stock No. 008083) can be used a control for this strain. This mutant mouse strain may be useful in studies of spinocerebellar ataxia 17 (SCA17) and polyglutamine (polyQ) related neurodegeneration.
A transgenic construct containing the human TATA box binding protein, TBP, containing a 71 polyQ repeat expansion, under the control of the mouse prion protein promoter was injected into fertilized FVB/N mouse eggs. Founder line TBP-71Q-16 was established. Male animals were bred to FVB/NJ mice. The mice were then crossed to B6CBAF1/J (Stock No. 100011) mice for 10 generations. The Donating Investigator reports that the strain did not thrive well enough to maintain on the FVB background.
Expressed Gene | TBP, TATA-box binding protein, human |
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Site of Expression |
Allele Name | transgene insertion 71-16, Xiao-Jiang Li |
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Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | SCA17; TBP-71Q-16 |
Gene Symbol and Name | Tg(Prnp-TBP*)71-16Xjl, transgene insertion 71-16, Xiao-Jiang Li |
Gene Synonym(s) | |
Promoter | Prnp, prion protein, mouse, laboratory |
Expressed Gene | TBP, TATA-box binding protein, human |
Strain of Origin | FVB/N |
Chromosome | UN |
Molecular Note | A transgenic construct containing the human TATA box binding protein, TBP, containing a 71 polyQ repeat expansion, under the control of the mouse prion protein promoter was injected into fertilized FVB/N mouse eggs. Founder line TBP-71Q-16 was established. The 71 polyQ-expansion is detected in the cerebral cortex and cerebellum by Western blot analysis. |
When maintaining a live colony on this F1 hybrid background, hemizygous mice are bred with B6CBAF1/J mice (Stock No. 100011). The Donating Investigator reports that the strain did not thrive well enough to maintain on the FVB background and bred male animals to female B6CBAF1/J mice. These TBP-71Q-16 transgenic mice have a reduced lifespan and begin to die at 5 months of age. Onset of progressive locomotor impairment is 7 weeks of age. The Donating Investigator has not attempted to make the strain homozygous.
When using the B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #008216 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Hemizygous or Non carrier for Tg(Prnp-TBP*)71-16Xjl |
Frozen Mouse Embryo | B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J Frozen Embryo | $3373.50 |
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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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