Strain Name: |
B6;C3-Tg(Prnp-SNCA*A53T)83Vle/J |
|---|---|
Stock Number: |
004479 |
Availability: | Repository- Live |
Price and Supply Information | |
General Terms and Conditions |
| Former Name |
B6;C3H-Tg(SNCA)83Vle/J (Changed: 21-MAR-08
) |
|
M83 (Changed: 15-DEC-04
) | |
| Genes & Alleles | Prnp; SNCA; Tg(Prnp-SNCA*A53T)83Vle; |
Product Information
Strain Details
Type JAX® GEMM® Strain - Mutant Stock Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Transgenic Mating System Hemizygote x Hemizygote (Female x Male) Species laboratory mouse Donating Investigator Virginia Lee, University of Pennsylvania Generation N?+N7F3 (06-DEC-07) Strain Description
Mice homozygous for the transgenic insert are viable and normal in size. These transgenic mice express human A53T variant alpha-synuclein (full-length, 140 amino acid isoform) under the direction of the mouse prion protein promoter. At 8 months of age, some homozygous mice develop a progressively severe motor phenotype. By 16 months of age, all homozygous mice display the phenotype. Lax grooming, weight loss and diminished mobility precede movement impairment, partial limb paralysis, trembling and inability to stand. Immunohistochemistry analysis of mutants between 8 to 12 months of age reveals widely distributed alpha-synuclein inclusions, with dense accumulation in the spinal cord, brainstem, cerebellum and thalamus. The appearance of alpha-synuclein aggregate inclusions parallels the onset of the motor impairment phenotype. Axons and myelin sheaths exhibit progressive ultrastructural degeneration. Immunoelectron microscopy and biochemical analysis show the inclusions in neurons are comprised primarily of 10-16 nm fibrils of alpha-synuclein. The structure, location and onset of the inclusions seen in the mutant mice resemble characteristics seen in human neuronal alpha-synucleinopathies, such as familial Parkinson's Disease. Mice hemizygous for the transgenic insert develop similar phenotypic traits, but onset occurslater, between 22 and 28 months of age. Homozygous mice have a high incidence of nonproductive matings. This mutant mouse strain represents a model that may be useful in studies of Parkinson's Disease.Strain Development
A transgenic construct containing the mouse prion protein promoter, its 5' and 3' untranslated regions and human alpha-synuclein A53T mutation cDNA sequence was injected into fertilized B6C3H mouse eggs. Transgenic animals are maintained on a mixed B6C3H background.
Related Disease (OMIM) Terms |
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Tg(Prnp-SNCA*A53T)83Vle | ||
|---|---|---|---|
| Allele Name | transgene insertion 83, Virginia M-Y Lee | ||
| Common Name(s) | A53T alpha-synuclein PRP; M83; Tg(SNCA)83Vle; | ||
| Mutation Made By | John Trojanowski, Dept. Pathology and Laboratory Med | ||
| Strain of Origin | C57BL/6 x C3H | ||
| Expressed Gene | SNCA, synuclein, alpha (non A4 component of amyloid precursor), human | ||
| Promoter | Prnp, prion protein, mouse, laboratory | ||
| General Note | This line was originally designated M83. Line M91 was also generated. | ||
| Molecular Note | The transgene contains the mouse prion protein promoter, its 5' and 3' untranslated regions, and a human alpha synuclein cDNA sequence encoding a mutated protein with an Ala53Thr mutation. Transgene expression was detected in the cerebral cortex, spinalcord, and cerebellum. [MGI Ref ID J:76657] | ||
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 100010 B6C3F1/J | (approximate) | |
| Considerations for Choosing Controls | ||
| Control Pricing Information for JAX® GEMM® Strains | ||
Genotyping Protocols
Tg(Prnp-SNCA*A53T)83Vle
Tg(Prnp-SNCA*A53T)83Vle QPCR
Colony Maintenance
| Breeding & Husbandry | The strain is maintained as a hemizygote on the same background. Homozygous mice have a high incidence of nonproductive matings. Coat color expected from breeding:Agouti. |
|---|---|
| Diet Information | LabDiet® 5K52/5K67 |
Related Strains
Strains carrying other alleles of Prnp
View Strains carrying other alleles of Prnp (18 strains)
008134 B6.Cg-Tg(THY1-SNCA*A30P)TS2Sud/J 008135 B6.Cg-Tg(THY1-SNCA*A53T)M53Sud/J 008389 C57BL/6-Tg(THY1-SNCA)1Sud/J
Additional Web Information
JAX® NOTES, Fall 2003; 491. Novel Familial Parkinson's Disease Model
JAX® NOTES, Summer 2007; 506. New Parkinson's Disease Mouse Model Resource.
Animal Health Reports
Room Number AX12
Research Applications
This mouse can be used to support research in many areas including:
Neurobiology Research
Neurodegeneration
Parkinson's Disease (synuclein mutants)
References
Selected Reference(s)
Additional ReferencesGiasson BI; Duda JE; Quinn SM; Zhang B; Trojanowski JQ; Lee VM. 2002. Neuronal alpha-synucleinopathy with severe movement disorder in mice expressing A53T human alpha-synuclein. Neuron 34(4):521-33. [PubMed: 12062037] [MGI Ref ID J:76657]
Price and Supply Information
| Strain Name: | B6;C3-Tg(Prnp-SNCA*A53T)83Vle/J |
| Stock Number: | 004479 |
Price Details
IMPORTANT NOTE: Prices are based on shipping destination. The shipping destinations are:
- USA, Canada and Mexico
- International
*Pricing for Shipping Destination selected:
International
| Price(s) in US dollars ($) | Genotype(s) Provided | ||||
|---|---|---|---|---|---|
| Individual Mouse Price | $307.40 | Hemizygous for Tg(Prnp-SNCA*A53T)83Vle | |||
| Individual Mouse Price | $307.40 | Homozygous for Tg(Prnp-SNCA*A53T)83Vle | |||
| Pair | $614.70 | Hemizygous for Tg(SNCA)83Vle x Hemizygous for Tg(SNCA)83Vle | |||
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. |
|---|---|
| Supply Notes |
Histology and Tissue Collection Services are available for all JAX® Mice strains. For more information, please contact Customer Service at orderquest@jax.org or 1-207-288-5845. Usually shipped between four and eight weeks of age. This strain is included in the Induced Mutant Resource Colony collection. Genomic DNA is available for this strain from the Mouse DNA Resource. |
| Licensing | See General Terms and Conditions below for Licensing and Use Restrictions |
| Control Information | View Control Information in Strain Details. View Control Pricing Information for JAX® Strains. |
General Terms and Conditions
View JAX® Mice & Services Conditions of Use.
For additional Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license prior to shipping.
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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