Strain Name:

B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J

Stock Number:

008216

Availability:

Cryopreserved - Ready for recovery

Use Restrictions Apply, see Terms of Use
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

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.
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Specieslaboratory mouse
GenerationN?pN1
 
Donating 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

View Strains carrying other alleles of Prnp     (20 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)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

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*)71-16Xjl/0

        FVB/N-Tg(Prnp-TBP*)71-16Xjl
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:130775)
    • mice have reduced lifespans relative to wild-type; mice start to die at 21 weeks of age
  • growth/size phenotype
  • decreased body size (MGI Ref ID J:130775)
    • symptomatic mice are visibly smaller than normal littermates at 6.5 months
  • behavior/neurological phenotype
  • abnormal motor coordination/ balance (MGI Ref ID J:130775)
    • onset of progressive motor impairment is 7 weeks of age
    • impaired coordination (MGI Ref ID J:130775)
      • mice show better initial performance relative to the other transgenic lines, then show a steady decline in performance
  • limb grasping (MGI Ref ID J:130775)
    • displayed by some mice
  • poor grooming (MGI Ref ID J:130775)
    • symptomatic mice can be distinguished from normal littermates at 6.5 months of age by poorly groomed appearance
  • sporadic seizures (MGI Ref ID J:130775)
    • some mice exhibit spontaneous seizures
  • tremors (MGI Ref ID J:130775)
    • displayed by some mice
  • nervous system phenotype
  • abnormal neuron morphology (MGI Ref ID J:130775)
    • TBP-71Q is relatively diffuse in neuronal nuclei in the brain
    • Purkinje cell degeneration (MGI Ref ID J:130775)
      • degenerating Purkinje cells are evident in cerebellum
    • abnormal neurite morphology (MGI Ref ID J:130775)
      • loss or disruption of calbindin-positive neurites in cerebellar molecular layer is observed in mutants
  • axon degeneration (MGI Ref ID J:130775)
    • 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
  • gliosis (MGI Ref ID J:130775)
    • gliosis is observed in granular and Purkinje cell layers of cerebellum
  • sporadic seizures (MGI Ref ID J:130775)
    • some mice exhibit spontaneous seizures
View Research Applications

Research Applications
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

 
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 OriginFVB/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]
 
 

Genotyping

Genotyping Information

Genotyping Protocols

(Prnp-TBP*)71-16Xjl, Standard PCR

Helpful Links

Genotyping resources and troubleshooting

References

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]

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

Colony Maintenance

Breeding & HusbandryWhen 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, General Terms & Conditions

Pricing

Pricing for USA, Canada and Mexico shipping destinations View International pricing
Price (US dollars $)
Cryorecovery Fee $1900.00
Animals Provided

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.

Additional Supply Details

Pricing for International shipping destinations View USA Canada and Mexico pricing
Price (US dollars $)
Cryorecovery Fee $2470.00
Animals Provided

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.

Additional Supply Details

Supply Details

Standard SupplyCryopreserved. 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).

  • 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
  USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
  International - Control Pricing Information for Genetically Engineered Mutant Strains.

Payment Terms and Conditions

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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.
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Terms of Use

Terms of Use


General Terms and Conditions


For Licensing and Use Restrictions view the link(s) below:
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