Search Criteria: Research Area is "Neurobiology Research: Ataxia (Movement) Defects (Spinocerebellar ataxia)"
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 008083 | B6CBA(FVB)-Tg(Prnp-TBP*)13Xjl/J | Repository- Live |
| 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 4 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). | ||
| 008216 | B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J | Repository- Live |
| 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(F
..... For more information please see the full descriiption on the strain data sheet | ||
| 003811 | B6D2-Tg(Pcp2SCA2)11Plt/J | Repository-Cryopreserved |
| These transgenic mice express the human SCA2 gene under the direction of the mouse Pcp2 promoter. Homozygous mice are viable and fertile. Transcripts and protein product are detected in the cerebellum. Immunohistochemical analysis indicates that the protein product is localized to the cytoplasm. Progressive functional deficits are evident as early as eight weeks of age as measured by hind leg clasping and stride length. Also noted is a dramatic loss of Purkinje cell arbor followed by a progressive decrease in Purkinje cell number. By 24-27 weeks, the number of Purkinje cells is reduced by approximately 50%. | ||
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Please indicate your interest in purchasing any of the strains listed below when they become available for distribution by checking the box next to the strain(s) of interest and then selecting the "Continue" button which leads to an Interest Form.View a Data sheet for New Strains Under Development
Select the strain name to link to the strain data sheet.
New Strains Under DevelopmentThe Jackson Laboratory serves as a worldwide distributor and national repository for common and rare strains of inbred mice and mice carrying spontaneous mutations or induced mutations (i.e., transgenic, targeted/"knockout", or chemically induced mutations). At any one time, we have over 100 strains at various stages of development and colony expansion. Strains "Under Development" fall into two categories depending on the anticipated demand from the scientific community.
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It is VERY IMPORTANT that you register interest in strains Under Development. The anticipated demand for a strain enables us to determine effectively the distribution plan for each strain Under Development. Registering interest also provides benefits to you (including advance notification of pending availability). Whether a strain is made available from a live colony OR from our cryopreservation repository, you may want to consider the option of Dedicated Supply. To learn more about Dedicated Supply, go to Services.
- Strains that will be made available from a live distribution colony at The Jackson Laboratory.
These strains are designated as: "Under Development for Distribution Colony"- Strains that will be made available through the Cryopreservation Repository.
These strains are designated as: "Under Development for Cryopreservation Repository"
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