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| These transgenic mice express a human TATA box binding protein, TBP, containing a 13 polyQ repeat expansion, under the control of the mouse prion protein promoter. These mice serve as a control strain for B6CBA(FVB)-Tg(Prnp-TBP*)105Xjl/J (Stock No. 008075) and B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J (Stock No. 008216). This mutant mouse strain may be useful in studies of spinocerebellar ataxia 17 (SCA17) and polyglutamine (polyQ) related neurodegeneration as a control strain. | |||||||||||||||
Type Mutant Stock; Transgenic; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Generation N10pN1 Donating Investigator Xiao-Jiang Li, Emory University School of Medicine Description
These transgenic mice express human TATA box binding protein, TBP, containing a 13 polyQ repeat expansion, under the control of the mouse prion protein promoter. Western blot analysis could not distinguish between endogenous and mutant TBP protein. Immunoreactive TBP protein was not detected in the brains of four month old mutants. Mice hemizygous for the 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 spinocerebellar ataxia 17 (SCA17) and polyglutamine (polyQ) related neurodegeneration. These mice serve as a control strain for B6CBA(FVB)-Tg(Prnp-TBP*)105Xjl/J (Stock No. 008075) and B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J (Stock No. 008216).Development
A transgenic construct containing the human TATA box binding protein, TBP, containing a 13 polyQ repeat expansion, under the control of the mouse prion protein promoter was injected into fertilized FVB/N mouse eggs. Founder line TBP-13Q 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.
| Control | ||
|---|---|---|
| Noncarrier | ||
| 100011 B6CBAF1/J | ||
| Considerations for Choosing Controls | ||
Strains carrying other alleles of Prnp
View Strains carrying other alleles of Prnp (20 strains)
Strains carrying other alleles of Tbp
008075 B6CBA(FVB)-Tg(Prnp-TBP*)105Xjl/J 008216 B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J View Strains carrying other alleles of Tbp (2 strains)
View Related Disease (OMIM) Terms
Related Disease (OMIM) Terms
NOT Spinocerebellar Ataxia 17; SCA17 - No similarity to the expected human disease phenotype was found.4
4 One or more human genes are associated with this human disease. The mouse genotype may involve mutations to orthologs of one or more of those genes, but the phenotype did not resemble the disease.
View Mammalian Phenotype Terms
Mammalian Phenotype Terms
assigned by genotype
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Tg(Prnp-TBP*)13Xjl/0
FVB/N-Tg(Prnp-TBP*)13Xjl
- life span-post-weaning/aging
- *normal* life span-post-weaning/aging (MGI Ref ID J:130775)
- mice do not show premature death as observed in other TBP transgenic lines
- behavior/neurological phenotype
- *normal* behavior/neurological phenotype (MGI Ref ID J:130775)
- mice do not display neurological phenotypes that seen in other TBP transgenic lines like motor abnormalities
- nervous system phenotype
- *normal* nervous system phenotype (MGI Ref ID J:130775)
- mice do not display phenotypes that seen in other TBP transgenic lines like neurodegeneration or inclusion bodies
| Allele Symbol | Tg(Prnp-TBP*)13Xjl | ||
|---|---|---|---|
| Allele Name | transgene insertion 13, Xiao-Jiang Li | ||
| Allele Type | Transgenic (random, expressed) | ||
| Common Name(s) | TBP-13Q; | ||
| 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 13 polyQ repeat expansion, under the control of the mouse prion protein promoter was injected into fertilized FVB/N mouse eggs. Founder line TBP-13Q was established. Western blot analysis could not distinguish between endogenous and mutant TBP protein. Immunoreactive TBP protein was not detected in 4 month old brains. [MGI Ref ID J:130775] | ||
Genotyping Protocols
(Prnp-TBP*)13Xjl, Standard PCR
Helpful Links
Genotyping resources and troubleshooting
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]
Colony Maintenance
Breeding & Husbandry When maintaining a live colony on this F1 hybrid background, hemizygous mice are bred with B6CBAF1/J mice (Stock No. 100011). The Donating Investigator has not attempted to make the strain homozygous.
| Pricing for USA, Canada and Mexico shipping destinations |
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Animals Provided
Price (US dollars $) Cryorecovery Fee $1900.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
| Pricing for International shipping destinations |
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Animals Provided
Price (US dollars $) Cryorecovery Fee $2470.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
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| Supply Notes |
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| Control | ||
|---|---|---|
| Noncarrier | ||
| 100011 B6CBAF1/J | ||
| Considerations for Choosing Controls | ||
| USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| International - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
Purchasing Information
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Surgical Services
Contact Information
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Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form
For Licensing and Use Restrictions view the link(s) below:
- Strain(s) not available to companies or for-profit entities.
| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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