Strain Name: |
B6.129S4-Tg(PSEN1H163R)G9Btla/J |
|---|---|
Stock Number: |
006469 |
Availability: | Repository- Live |
General Terms and Conditions |
| Genes & Alleles | PSEN1; Tg(PSEN1H163R)G9Btla; |
Product Information
Strain Details
Type JAX® GEMM® Strain - Congenic Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Mutant Strain Type JAX® GEMM® Strain - Transgenic Mating System Hemizygote x +/+ sibling (Female x Male) Species laboratory mouse Donating Investigator Bruce Lamb, The Cleveland Clinic Foundation Generation ?+N34+F1 (20-DEC-07) Strain Description
Mice hemizygous for this "H163R mutant PSEN1 YAC" transgene are viable and fertile, while the donating investigator reports that homozygous mice are non-viable. Semi-quantitative RT-PCR of multiple tissues shows expression of H163R mutant human PSEN1 at levels comparable to that of wildtype mouse PSEN1 (50–70%). In contrast to other PSEN1 transgenic models, tissues from this strain express alternatively spliced human PSEN1 transcripts encoding PSEN1 protein (with or without the tetrapeptide VRSQ) and accumulated an 18-kDa PSEN1 C-terminal fragment as shown by western blots, thus expressing a wide spectrum of different human PSEN1 mRNAs and proteins. When crossed to other FAD transgenic strains (for example Stock No. 005300), this transgene is associated with elevated levels of the 42 amino acid form of amyloid-beta (1–42) in both brain and plasma. These mice may be useful in studying neurological disorders such as Familial Alzheimer’s Disease and Down syndrome.Strain Development
A 1000 kb YAC transgene (788H12) containing the entire human presenilin-1 (PSEN or PS-1) gene was altered to include the H163R PSEN1 Familial Alzheimer's Disease (FAD) mutation. The transgene also included approximately 550 kb of upstream and 350 kb of downstream flanking sequences. This construct was injected into 129S4/SvJae-derived J1 embryonic stem (ES) cells. Founder animals (line G9) were backcrossed to C57BL/6 for at least 33 generations.
Related Disease (OMIM) Terms |
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Tg(PSEN1H163R)G9Btla | ||
|---|---|---|---|
| Allele Name | transgene insertion G9, Bruce Lamb | ||
| Common Name(s) | (H163R) PS-1 YAC; H163R mutant PS-1 YAC; H163R mutant PS-1 YAC transgenic mouse (line G9); PS-1 YAC transgenic line G9; | ||
| Mutation Made By | Bruce Lamb, The Cleveland Clinic Foundation | ||
| Strain of Origin | 129S4/SvJae | ||
| ES Cell Line Name | J1 | ||
| ES Cell Line Strain | 129S4/SvJae | ||
| Expressed Gene | PSEN1, presenilin 1 (Alzheimer disease 3), human | ||
| Promoter | PSEN1, presenilin 1 (Alzheimer disease 3), human | ||
| General Note | The transgene construct was injected into embryonic stem (ES) cells. | ||
| Molecular Note | A 1000 kb YAC transgene (788H12) containing the entire human presenilin-1 gene was altered to include the Familial Alzheimer's Disease- (FAD-) associated histidine-to-arginine substitution at amino acid position 163 (H163R). The transgene also includes approximately 550 kb of upstream and 350 kb of downstream flanking DNA. Semi-quantitative RT-PCR of multiple tissues shows expression of H163R mutant human mRNA at 50-70% of the level of wild type mouse transcripts. Transgenic tissues express a wide spectrum of human presenilin-1 mRNA and protein species; alternatively spliced human mRNAs encoding presenilin 1, with or without the tetrapeptide VRSQ, are detected, and western blot analysis reveals accumulation of an 18-kDa C-terminal peptide. [J:58050] | ||
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Genotyping Protocols
Tg(PSEN1H163R)G9Btla
Colony Maintenance
| Breeding & Husbandry | When maintaining a live colony, hemizygous mice are bred to wildtype siblings or to C57BL/6J inbred mice. The donating investigator reports that homozygous mice are non-viable. |
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| Diet Information | LabDiet® 5K52/5K67 |
Related Strains
Strains carrying other alleles of PSEN1
View Strains carrying other alleles of PSEN1 (5 strains)
Additional Web Information
Congenic Nomenclature
Visit the Alzheimer's Disease Mouse Model Resource site for helpful information on Alzheimer's Disease and research resources.
Animal Health Reports
Room Number AX11
Research Applications
This mouse can be used to support research in many areas including:
Mouse/Human Gene Homologs
Alzheimer's
Neurobiology Research
Alzheimer's Disease (Presenilin mutants)
Alzheimer's Disease
Neurodegeneration
Research Tools
Neurobiology Research
References
Selected Reference(s)
Lamb BA; Bardel KA; Kulnane LS; Anderson JJ; Holtz G; Wagner SL; Sisodia SS; Hoeger EJ. 1999. Amyloid production and deposition in mutant amyloid precursor protein and presenilin-1 yeast artificial chromosome transgenic mice. Nat Neurosci 2(8):695-7. [PubMed: 10412057] [J:58050]
Price and Supply Information
| Strain Name: | B6.129S4-Tg(PSEN1H163R)G9Btla/J |
| Stock Number: | 006469 |
Price Details
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Supply Details
| Standard Supply | Repository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement. |
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| Supply Notes |
Usually shipped between four and eight weeks of age. This strain is included in the Induced Mutant Resource Colony collection. |
| Licensing | See General Terms and Conditions below for Licensing and Use Restrictions |
| Control Information | View Control Information in Strain Details. |
General Terms and Conditions
View JAX® Mice & Services Conditions of Use.
For additional Licensing and Use Restrictions view the link(s) below:
- Strain(s) not available to companies or for-profit entities.
The Jackson Laboratory's Genotype Promise
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.Ordering and Purchasing Information
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