Strain Name: |
FVB-Tg(ACTA1-PABPN1*A17)1Drub/J |
|---|---|
Stock Number: |
006655 |
Availability: | Repository- Live |
General Terms and Conditions |
| Former Name |
FVB-Tg(ACTA1-PABPN1*17)1Drub/J (Changed: 11-JAN-07
) |
| Genes & Alleles | ACTA1; PABPN1; Tg(ACTA1-PABPN1*A17)1Drub; |
Product Information
Strain Details
Type JAX® GEMM® Strain - Coisogenic Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Mutant Strain Type JAX® GEMM® Strain - Transgenic Mating System +/+ sibling x Hemizygote (Female x Male) Species laboratory mouse Donating Investigator David Rubinsztein, Cambridge Institute for Medical Research Generation N1+1F1 (12-DEC-07) Strain Description
Mice hemizygous for the transgenic insert are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities until roughly four months of age. At four months, hemizygotes develop a time-dependent progressive muscle weakness (measured by grip strength, wire maneuver and vertical gripping tests), which leads to late onset locomoter defects around 9 months of age. At 9 months mice can not lift their own body weight. They drag their pelvis when walking and show reluctance to walk. There is no difference in body weight or mortality up to 15 months of age compared to controls. Hemizygotes develop KCl-insoluble inclusions containing PABPN1 in the nuclei of skeletal muscle fibers with tubulo-filamentous ultrastructures. The proportion of myocte nuclei with aggregates increases with age. Significantly elevated numbers of TUNEL-positive myocyte nuclei can be found at 6 and 12 months. TUNEL staining is widely used as a cell-death marker in muscle diseases in mice and humans. Muscles of hemizygotes contain increased numbers of centrally located nuclei and vacuoles compared to controls, which reflects the regenerative processes that can result from muscular dystrophy. This strain my prove useful as a model of human Oculopharyngeal Muscular Dystrophy and in Muscular Dystrophy or Codon Reiteration Disease research.Strain Development
A transgenic construct containing a human skeletal actin (HSA1) promoter upstream of a bovine poly-(A) binding protein nuclear 1 (PABPN1) with 17 alanines was injected into FVB donor eggs.
Related Disease (OMIM) Terms |
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Tg(ACTA1-PABPN1*A17)1Drub | ||
|---|---|---|---|
| Allele Name | transgene insertion 1, David Rubinsztein | ||
| Common Name(s) | A17-1; | ||
| Strain of Origin | FVB/N | ||
| Expressed Gene | PABPN1, poly(A) binding protein, nuclear 1, human | ||
| Promoter | ACTA1, actin, alpha 1, skeletal muscle, human | ||
| General Note | Of two lines descended from founders with this transgenic construct, line A7-1 has a more severe phenotype, correlated with higher transgene expression levels, than line A7-2. | ||
| Molecular Note | The transgene contains the human skeletal actin promoter upstream of a mutant bovine cDNA with a series of CGC codons encoding a 17-amino acid polyalanine tract. [MGI Ref ID J:115642] | ||
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 001800 FVB/NJ | ||
| Considerations for Choosing Controls | ||
Genotyping Protocols
Tg(ACTA1-PABPN1*A17)1Drub
Colony Maintenance
| Diet Information | LabDiet® 5K52/5K67 |
|---|
Related Strains
Strains carrying other alleles of ACTA1
View Strains carrying other alleles of ACTA1 (7 strains)
Animal Health Reports
Room Number AX11
Research Applications
This mouse can be used to support research in many areas including:
Neurobiology Research
Neuromuscular Defects
References
Selected Reference(s)
Additional ReferencesDavies JE; Wang L; Garcia-Oroz L; Cook LJ; Vacher C; O'Donovan DG; Rubinsztein DC. 2005. Doxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic mice. Nat Med 11(6):672-7. [PubMed: 15864313] [MGI Ref ID J:115642]
Price and Supply Information
| Strain Name: | FVB-Tg(ACTA1-PABPN1*A17)1Drub/J |
| Stock Number: | 006655 |
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. |
|---|---|
| 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 |
| Control Information | View Control Information in Strain Details. |
General Terms and Conditions
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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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