These αB-crys Z104 mice express a transgene containing human alpha B crystallin under the direction of the mouse prion protein promoter and may be useful in studies of amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease), protein aggregation and neuronal proteostasis.
Dr. David R Borchelt, University of Florida
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence, Humanized sequence) |
The small heat-shock protein family member alpha B crystallin binds misfolded proteins and holds them in large soluble aggregates.
These transgenic mice express the human alpha B crystallin under the direction of the mouse prion protein promoter, producing approximately 6-fold overexpression of alpha beta crystallin (mouse and human) in the spinal cord, approximately 30-fold overexpression in the forebrain, and high levels of expression also detected in the cerebellum by Western blot analysis. Transgenic mice exhibit 20% more motor neurons in the spinal cord when compared to wildtype controls.
Mice that are hemizygous for the transgene are viable and fertile. The Donating Investigator has not attempted to make the strain homozygous.
A transgenic construct containing cDNA of human alpha B crystallin under the control of the mouse Prnp, prion protein, gene promoter, was injected into fertilized C3/B6 F2 mouse eggs. Upon arrival, sperm was cryopreserved. To establish our live colony, an aliquot of frozen sperm was used to fertilize B6C3F1/J oocytes (Stock No. 100010).
Expressed Gene | CRYAB, crystallin alpha B, human |
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Site of Expression | Human alpha B crystallin is expressed in the forebrain, cerebellum, spinal cord and kidney. |
Allele Name | transgene insertion Z104, David R Borchelt |
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Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | Z104 |
Gene Symbol and Name | Tg(Prnp-CRYAB)Z104Dbo, transgene insertion Z104, David R Borchelt |
Gene Synonym(s) | |
Promoter | Prnp, prion protein, mouse, laboratory |
Expressed Gene | CRYAB, crystallin alpha B, human |
Site of Expression | Human alpha B crystallin is expressed in the forebrain, cerebellum, spinal cord and kidney. |
Strain of Origin | (C3H/HeJ x C57BL/6J)F2 |
Chromosome | UN |
Molecular Note | The transgenic construct contains cDNA of human alpha B crystallin under the control of the mouse Prnp, prion protein, gene promoter. The 6 kb promoter consists of the first exon, the first intron, and the fused 2nd and 3rd exons. Expression of the transgene is observed in the forebrain, cerebellum, spinal cord and kidney. No evidence of expression was found in lung, spleen, testes or liver. |
When maintaining a live colony, hemizygous mice may be bred to wildtype siblings, or to B6C3F1/J mice (Stock No. 100010). The Donating Investigator has not attempted to make the strain homozygous.
When using the αB-crys Z104 mouse strain in a publication, please cite the originating article(s) and include JAX stock #027513 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Hemizygous or Non carrier for Tg(Prnp-CRYAB)Z104Dbo |
Frozen Mouse Embryo | B6;C3-Tg(Prnp-CRYAB)Z104Dbo/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6;C3-Tg(Prnp-CRYAB)Z104Dbo/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6;C3-Tg(Prnp-CRYAB)Z104Dbo/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | B6;C3-Tg(Prnp-CRYAB)Z104Dbo/J Frozen Embryo | $3373.50 |
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