Strain Name:

B6CBA-Tg(Prnp-TBP*)105Xjl/J

Stock Number:

008075

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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. Hemizygotes exhibit weight loss, kyphosis, locomotor impairment, nuclear inclusions of polyQ tracts in neurons, gliosis and neuron loss. This transgenic strain has a more severe phenotype and earlier onset of 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.

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

Type Mutant Stock; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
GenerationN?pN1
Generation Definitions
 
Donating Investigator Xiao-Jiang Li,   Emory University School of Medicine

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.

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.

Control Information

  Control
   Noncarrier
   100011 B6CBAF1/J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Prnp
012938   129-Prnptm2Edin/J
016925   129;B6-Grin3b/Tmem259tm1Zhang Tg(Prnp-C19ORF6,-GFP)6Zhang/J
003960   129S6-Tg(Prnp-GFP/cre)1Blw/J
005866   B6.Cg-Tg(APP695)3Dbo Tg(PSEN1dE9)S9Dbo/Mmjax
006006   B6.Cg-Tg(Prnp-APP)A-2Dbo/J
008596   B6.Cg-Tg(Prnp-Abca1)EHol/J
006005   B6.Cg-Tg(Prnp-App/APPswe)E1-2Dbo/Mmjax
007180   B6.Cg-Tg(Prnp-ITM2B/APP695*40)1Emcg/J
007182   B6.Cg-Tg(Prnp-ITM2B/APP695*42)A12Emcg/J
006823   B6.Cg-Tg(Prnp-SNCA*A53T)23Mkle/J
010700   B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J
009337   B6.FVB-Tg(Prnp-RTN3)2Yanr/J
007002   B6;C3-Tg(Prnp-ITM2B/APP695*42)A12Emcg/Mmjax
008169   B6;C3-Tg(Prnp-MAPT*P301S)PS19Vle/J
004479   B6;C3-Tg(Prnp-SNCA*A53T)83Vle/J
018917   B6;SJL-Tg(Prnp-CCS)17Jlel/J
003378   B6C3-Tg(APP695)3Dbo Tg(PSEN1)5Dbo/J
008216   B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J
008083   B6CBA-Tg(Prnp-TBP*)13Xjl/J
003741   B6D2-Tg(Prnp-MAPT)43Vle/J
024841   B6N.Cg-Tg(Prnp-MAPT*P301S)PS19Vle/J
017907   B6N.Cg-Tg(Prnp-TARDBP)96Dwc/J
017933   B6N.Cg-Tg(Prnp-TARDBP*Q331K)103Dwc/J
017930   B6N.Cg-Tg(Prnp-TARDBP*Q331K)109Dwc/J
025402   B6SJL-Tg(Prnp-Immt/SOD1)1Gmnf/J
025403   B6SJL-Tg(Prnp-Immt/SOD1*G93A)7Gmnf/J
016201   B6SJL-Tg(Prnp-TARDBP)4Jlel/J
016203   B6SJL-Tg(Prnp-TARDBP*A315T)23Jlel/J
016608   C57BL/6-Tg(Prnp-TARDBP)3cPtrc/J
017604   C57BL/6-Tg(Prnp-TARDBP*M337V)4Ptrc/J
019517   FVB-Tg(Prnp-HSPB1)1Kolb/J
019482   FVB-Tg(Prnp-HSPB1*R136W)1Kolb/J
018122   FVB.129S7(B6)-Prnptm1Cwe/J
017678   FVB;129-Pink1tm1Aub Tg(Prnp-SNCA*A53T)AAub/J
017744   FVB;129-Tg(Prnp-SNCA*A53T)AAub/J
017916   STOCK Tg(Prnp-FUS)WT3Cshw/J
016144   STOCK Tg(Prnp-TARDBP)4Jlel/J
016143   STOCK Tg(Prnp-TARDBP*A315T)23Jlel/J
008212   STOCK Tg(SMN2)89Ahmb Smn1tm1Msd Tg(Prnp-SMN)92Ahmb/J
View Strains carrying other alleles of Prnp     (39 strains)

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

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.
Spinocerebellar Ataxia 17; SCA17
- Potential model based on transgenic expression of an ortholog of a human gene that is associated with this disease. Phenotypic similarity to the human disease has not been tested.
Parkinson Disease, Late-Onset; PD   (TBP)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Tg(Prnp-TBP*)105Xjl/0

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

 
Allele Symbol Tg(Prnp-TBP*)105Xjl
Allele Name transgene insertion 105, Xiao-Jiang Li
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) TBP-105Q;
Strain of OriginFVB/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 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]
 
 

Genotyping

Genotyping Information

Genotyping Protocols

(Prnp-TBP*)105Xjl, 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, G200.

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-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.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2140.00
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.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. 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. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 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 View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2782.00
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.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. 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. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 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).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

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 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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