B6CBA-Tg(Prnp-TBP*)13Xjl/J

Stock number: 008083

Description

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.

Detailed 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.

Allele

Symbol: Tg(Prnp-TBP*)13Xjl
Name: transgene insertion 13, Xiao-Jiang Li
MGI accession ID: MGI:3772701
Generation method: Transgenic
Attributes: Inserted expressed sequence; Humanized sequence
Marker symbol: Tg(Prnp-TBP*)13Xjl
Marker name: transgene insertion 13, Xiao-Jiang Li
Chromosome: UN
Strain of origin: FVB/N
Expressed genes: TBP,TATA-box binding protein,human
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.