Strain Name: |
B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J |
|---|---|
Stock Number: |
008216 |
Availability: | Repository- Live |
General Terms and Conditions |
| Former Name |
B6CBA(FVB)-Tg(Prnp-TBP*)71Q16Xjl/J (Changed: 06-MAR-08
) |
| Genes & Alleles | Prnp; Tbp; Tg(Prnp-TBP*)71-16Xjl; |
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 Shihua Li, Emory University School of Medicine Generation N11+ (10-JUN-08) Strain Description
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.Strain Development
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.)
Related Disease (OMIM) Terms |
Mammalian Phenotype Terms assigned by genotype |
| Allele Symbol | Tg(Prnp-TBP*)71-16Xjl | ||
|---|---|---|---|
| Allele Name | transgene insertion 71-16, Xiao-Jiang Li | ||
| Common Name(s) | TBP-71Q-16; | ||
| 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 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. [MGI Ref ID J:130775] | ||
| Control | ||
|---|---|---|
| Noncarrier | ||
| 100011 B6CBAF1/J | ||
| Considerations for Choosing Controls | ||
| 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-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. |
|---|---|
| Diet Information | LabDiet® 5K52/5K67 |
Strains carrying other alleles of Prnp
View Strains carrying other alleles of Prnp (18 strains)
Strains carrying other alleles of Tbp
008075 B6CBA(FVB)-Tg(Prnp-TBP*)105Xjl/J 008083 B6CBA(FVB)-Tg(Prnp-TBP*)13Xjl/J View Strains carrying other alleles of Tbp (2 strains)
Room Number AX11
Neurobiology Research
Ataxia (Movement) Defects (Spinocerebellar ataxia)
Neurodegeneration
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]
| Strain Name: | B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J |
| Stock Number: | 008216 |
IMPORTANT NOTE: Prices are based on shipping destination. To view prices, select your shipping destination.
| 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. |
|---|---|
| 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. |
Purchasing Information
JAX® Mice Orders
Surgical Services
Contact Information
Orders & Technical Support
Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form