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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 SystemNoncarrier x Hemizygote         (Female x Male)
Specieslaboratory mouse
Donating Investigator Xiao-Jiang Li,   Emory University School of Medicine
GenerationN11+ (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

Spinocerebellar Ataxia 17; SCA17
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*)105Xjl/0

        FVB/N-Tg(Prnp-TBP*)105Xjl
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:130775)
    • mice have reduced lifespans relative to wild-type; mice start to die as early as 9 weeks of age
  • growth/size phenotype
  • decreased body size (MGI Ref ID J:130775)
    • symptomatic mice are visibly smaller than normal littermates at 3 months
    • weight loss (MGI Ref ID J:130775)
      • mice begin to lose body weight at 8 weeks of age
  • behavior/neurological phenotype
  • abnormal motor coordination/ balance (MGI Ref ID J:130775)
    • onset of progressive motor impairment is 6 weeks of age
    • impaired coordination (MGI Ref ID J:130775)
      • mice perform poorly on a non-accelerating rotating rod at 6 weeks of age, and do not show any subsequent improvement
  • 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 3 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
  • 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
  • neuron degeneration (MGI Ref ID J:130775)
    • degenerating neurons are detected in granular layer of cerebellum
  • neuronal intranuclear inclusions (MGI Ref ID J:130775)
    • prominent nuclear inclusions form in cerebellar granule neurons
  • sporadic seizures (MGI Ref ID J:130775)
    • some mice exhibit spontaneous seizures
  • skeleton phenotype
  • kyphosis (MGI Ref ID J:130775)
    • posture is conspicuously abnormal in mutants; kyphosis, indicative of proximal muscle weakness, is observed by 3 months of age

Gene & Allele Details

Allele Symbol Tg(Prnp-TBP*)105Xjl
Allele Name transgene insertion 105, Xiao-Jiang Li
Common Name(s) TBP-105Q;
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 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 & HusbandryWhen 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.
Diet Information LabDiet® 5K52/5K67

Related Strains

View Strains carrying other alleles of Prnp     (18 strains)

Strains carrying other alleles of Tbp
008083   B6CBA(FVB)-Tg(Prnp-TBP*)13Xjl/J
008216   B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J
View Strains carrying other alleles of Tbp     (2 strains)

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 SupplyRepository-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.
LicensingSee General Terms and Conditions below  
Control InformationView Control Information in Strain Details.

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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.
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