Strain Name: |
B6CBA(FVB)-Tg(Prnp-TBP*)105Xjl/J |
|---|---|
Stock Number: |
008075 |
Availability: | Repository- Live |
General Terms and Conditions |
| Genes & Alleles | Prnp; Tbp; Tg(Prnp-TBP*)105Xjl; |
Product Information
Strain Details
Type JAX® GEMM® Strain - Mutant Stock Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Transgenic Mating System Noncarrier x Hemizygote (Female x Male) Species laboratory mouse Donating Investigator Xiao-Jiang Li, Emory University School of Medicine Generation N11+ (16-JUN-08) Strain Description
These transgenic mice express a human TATA box binding protein, TBP, containing a 105 polyQ repeat expansion, under the control of the mouse prion protein promoter. The 105 polyQ-expansion is detected by Western blot analysis. At 3 months of age, hemizygous transgenic mice are smaller than wildtype littermates, appear ungroomed, exhibit kyphosis and weight loss. These transgenic mice have a shortened lifespan of 5 months and begin to die as early as 9 weeks of age. Immunohistochemical and Western blot analysis of brain tissue reveals neuronal nuclear aggregates of polyQ tracts by 8 weeks of age. Electron microscopic examination of brain tissue shows nuclear inclusions in cerebellar granule neurons and degeneration of Purkinje cells and axons. Reactive gliosis is observed in the granular and Purkinje layers. Loss of Purkinje cells is observed at 10 weeks of age. Apoptopic neurons in brain cortex and spinal cord are more abundant in transgenic mice when compared to wildtype. Early neurodegeneration is observed in the cerebral cortex. Onset of progressive locomotor impairment is 6 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 more severe phenotype than B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J (Stock No. 008216). 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.Strain Development
A transgenic construct containing the human TATA box binding protein, TBP, containing a 105 polyQ repeat expansion, under the control of the mouse prion protein promoter was injected into fertilized FVB/N mouse eggs. Founder line TBP-105Q was established from a female animal. The founder female was bred to a FVB/NJ male. 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.)
Related Disease (OMIM) Terms |
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Tg(Prnp-TBP*)105Xjl | ||
|---|---|---|---|
| Allele Name | transgene insertion 105, Xiao-Jiang Li | ||
| Common Name(s) | TBP-105Q; | ||
| Strain of Origin | FVB/N | ||
| Expressed Gene | Tbp, TATA box binding protein, mouse, laboratory | ||
| Promoter | Prnp, prion protein, mouse, laboratory | ||
| Molecular Note | A transgenic construct containing the human TATA box binding protein, TBP, containing a 105 polyQ repeat expansion, under the control of the mouse prion protein promoter was injected into fertilized FVB/N mouse eggs. Founder line TBP-105Q was established. The 105 polyQ-expansion was detected in the cerebral cortex and cerebellum by Western blot analysis. [MGI Ref ID J:130775] | ||
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 100011 B6CBAF1/J | ||
| Considerations for Choosing Controls | ||
Colony Maintenance
| Breeding & Husbandry | When maintaining a live colony, these mice are bred as hemizygotes. 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-105Q transgenic mice have a reduced lifespan of 5 months and begin to die as early as 9 weeks of age. Onset of progressive locomotor impairment is 6 weeks of age. The donating investigator has not attempted to make the strain homozygous. |
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| Diet Information | LabDiet® 5K52/5K67 |
Related Strains
Strains carrying other alleles of Prnp
View Strains carrying other alleles of Prnp (18 strains)
008083 B6CBA(FVB)-Tg(Prnp-TBP*)13Xjl/J 008216 B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J
Animal Health Reports
Room Number AX12
Research Applications
This mouse can be used to support research in many areas including:
Neurobiology Research
Ataxia (Movement) Defects (Spinocerebellar ataxia)
Neurodegeneration
References
Selected Reference(s)
Friedman MJ; Shah AG; Fang ZH; Ward EG; Warren ST; Li S; Li XJ. 2007. Polyglutamine domain modulates the TBP-TFIIB interaction: implications for its normal function and neurodegeneration. Nat Neurosci 10(12):1519-28. [PubMed: 17994014] [MGI Ref ID J:130775]
Price and Supply Information
| Strain Name: | B6CBA(FVB)-Tg(Prnp-TBP*)105Xjl/J |
| Stock Number: | 008075 |
Price Details
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Supply Details
| Standard Supply | Repository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement. |
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| Supply Notes |
Usually shipped between four and eight weeks of age. This strain is included in the Induced Mutant Resource Colony collection. |
| Licensing | See General Terms and Conditions below |
| Control Information | View Control Information in Strain Details. |
General Terms and Conditions
View JAX® Mice & Services Conditions of Use.
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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.Ordering and Purchasing Information
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