B6;C3-Tg(Prnp-CRYAB)Z104Dbo/J

Stock number: 027513
Product name: αB-crys Z104
Research areas: Neurobiology Research, Research Tools

Description

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.

Detailed description

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.

Development

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).

Allele

Symbol: Tg(Prnp-CRYAB)Z104Dbo
Name: transgene insertion Z104, David R Borchelt
MGI accession ID: MGI:5697709
Generation method: Transgenic
Attributes: Inserted expressed sequence; Humanized sequence
Marker symbol: Tg(Prnp-CRYAB)Z104Dbo
Marker name: transgene insertion Z104, David R Borchelt
Chromosome: UN
Strain of origin: (C3H/HeJ x C57BL/6J)F2
Expressed genes: CRYAB,crystallin alpha B,human
Site of expression: Human alpha B crystallin is expressed in the forebrain, cerebellum, spinal cord and kidney.
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.