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| 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. | |||||||||||||||||||
- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Strain Information
Former Names B6CBA(FVB)-Tg(Prnp-TBP*)71Q16Xjl/J (Changed: 06-MAR-08 ) Type Mutant Stock; Transgenic; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Generation N?pN1
Generation DefinitionsDonating Investigator Shihua Li, Emory University School of Medicine 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.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.
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 100011 B6CBAF1/J | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying other alleles of Prnp
View Strains carrying other alleles of Prnp (21 strains)
008075 B6CBA(FVB)-Tg(Prnp-TBP*)105Xjl/J 008083 B6CBA(FVB)-Tg(Prnp-TBP*)13Xjl/J
Phenotype
Phenotype Information
assigned by genotype
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Tg(Prnp-TBP*)71-16Xjl/0
FVB/N-Tg(Prnp-TBP*)71-16Xjl
- mortality/aging
- premature death
- mice have reduced lifespans relative to wild-type; mice start to die at 21 weeks of age (MGI Ref ID J:130775)
- growth/size phenotype
- decreased body size
- symptomatic mice are visibly smaller than normal littermates at 6.5 months (MGI Ref ID J:130775)
- weight loss (MGI Ref ID J:130775)
- behavior/neurological phenotype
- abnormal motor coordination/ balance
- onset of progressive motor impairment is 7 weeks of age (MGI Ref ID J:130775)
- impaired coordination
- mice show better initial performance relative to the other transgenic lines, then show a steady decline in performance (MGI Ref ID J:130775)
- limb grasping
- displayed by some mice (MGI Ref ID J:130775)
- poor grooming
- symptomatic mice can be distinguished from normal littermates at 6.5 months of age by poorly groomed appearance (MGI Ref ID J:130775)
- sporadic seizures
- some mice exhibit spontaneous seizures (MGI Ref ID J:130775)
- tremors
- displayed by some mice (MGI Ref ID J:130775)
- nervous system phenotype
- abnormal neuron morphology
- TBP-71Q is relatively diffuse in neuronal nuclei in the brain (MGI Ref ID J:130775)
- axon degeneration
- degenerating axons are evident in cerebellum; axons with reduced internal space surrounded by a distorted or thickened myelin sheath, presence of myelin ovoids, or vacuolated axons without distinguishable organelles or disintegrating myelin sheaths are indicative of more severe degeneration (MGI Ref ID J:130775)
- gliosis
- gliosis is observed in granular and Purkinje cell layers of cerebellum (MGI Ref ID J:130775)
- sporadic seizures
- some mice exhibit spontaneous seizures (MGI Ref ID J:130775)
This mouse can be used to support research in many areas including:
Neurobiology Research
Ataxia (Movement) Defects
Spinocerebellar ataxia
Neurodegeneration
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | Tg(Prnp-TBP*)71-16Xjl | ||
|---|---|---|---|
| Allele Name | transgene insertion 71-16, Xiao-Jiang Li | ||
| Allele Type | Transgenic (random, expressed) | ||
| Common Name(s) | TBP-71Q-16; | ||
| Strain of Origin | FVB/N | ||
| Expressed Gene | TBP, TATA box binding protein, human | ||
| 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] | ||
Genotyping
Genotyping Information
Genotyping Protocols
(Prnp-TBP*)71-16Xjl, Standard PCR
Helpful Links
Genotyping resources and troubleshooting
References
References provided by MGI
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]
Health & husbandry
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Health & Colony Maintenance Information
Animal Health Reports
Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, RG10/RG30.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 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.
Purchasing information
Pricing, Supply Level & Notes, Controls
| Pricing for USA, Canada and Mexico shipping destinations |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $1980.00 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.
Supply Notes
- Cryorecovery - Standard.
We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 13 and 16 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
| Pricing for International shipping destinations |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2574.00 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.
Supply Notes
- Cryorecovery - Standard.
We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 13 and 16 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
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Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
General Supply Notes
- This strain is included in the Induced Mutant Resource Colony collection.
- Genomic DNA is available for this strain from the Mouse DNA Resource.
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 100011 B6CBAF1/J | ||
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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The Jackson Laboratory's Genotype Promise
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 Information
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For Licensing and Use Restrictions view the link(s) below:
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Contact information
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| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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