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| In these trangenic mice, widespread overexpression of the mitochondrial ribosomal RNA methyltransferase, mtTFB1, results in maternally inherited deafness. | |||||||||||||||||||
- Description
- Disease & phenotype
- Genes & alleles
- Genotyping
- Health & care
- References
- Pricing & purchasing
- Terms of use
Description
Strain Information
Type Congenic; Mutant Strain; Transgenic; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Mating System Noncarrier x Hemizygote (Female x Male) 28-AUG-12 Mating System Hemizygote x Noncarrier (Female x Male) 28-AUG-12 Species laboratory mouse Generation N8pN1+F1 (28-AUG-12)
Generation DefinitionsDonating Investigator Gerald S Shadel, Yale University Description
These CAG-Tfb1m mice possess the CMV enhancer/chicken beta-actin/rabbit beta-globin hybrid promoter driving ubiquitous expression of the mitochondrial transcription factor B1 (Tfb1m) gene. TFB1M is a dimethyltransferase which acts specifically on mitochondrial 12S rRNA at a conserved stem loop at the 3-prime end of the molecule and is necessary for mitochondrial ribosome biogenesis and translation. Over-expression of human TFB1M in cultured cells results in cellular and molecular phenotypes similar to those in cell containing the A1555G point mutation in human mtDNA that causes maternally inherited deafness. These mice exhibit progressive hearing loss due to apoptosis of spiral ganglion neurons and stria vascularis in the inner ear. Hemizygous mice are viable, fertile, and normal in size.Development
The CAG-Tfb1m transgene was designed with the CMV enhancer/chicken beta-actin/rabbit beta-globin hybrid promoter driving expression of the mitochondrial transcription factor B1 (Tfb1m) gene. The construct was microinjected into C57BL/6J x SJL/J embryos, and mice from founder line A were bred to C57BL/6J mice to establish a colony. These mice were backcrossed at least 8 generations to C57BL/6J mice. Upon arrival at The Jackson Laboratory, mice were bred to C57BL/6J inbred mice (Stock No. 000664) for at least one generation.
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 000664 C57BL/6J | (approximate) | |
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying other alleles of CMV
View Strains carrying other alleles of CMV (36 strains)
Phenotype
Phenotype Information
This mouse can be used to support research in many areas including:
Mouse/Human Gene Homologs
deafness
Neurobiology Research
Hearing Defects
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | Tg(CAG-Tfb1m)AGsha | ||
|---|---|---|---|
| Allele Name | transgene insertion A, Gerald Shadel | ||
| Allele Type | Transgenic (random, expressed) | ||
| Common Name(s) | CAG-Tfb1m; Tg-mtTFB1; | ||
| Mutation Made By | Gerald Shadel, Yale University | ||
| Strain of Origin | C57BL/6J x SJL/J | ||
| Expressed Gene | Tfb1m, transcription factor B1, mitochondrial, mouse, laboratory | ||
| Promoter | CMV, cytomegalovirus, human | ||
| Molecular Note | The CAG-Tfb1m transgene was designed with the CMV enhancer/chicken beta-actin/rabbit beta-globinhybrid promoter driving expression of the mitochondrial transcription factor B1 (Tfb1m) gene. Line A was established. [MGI Ref ID J:181548] | ||
Genotyping
Genotyping Information
Genotyping Protocols
Tg (CAG-Tfb1m)1Gsha, Separated PCR
Helpful Links
Genotyping resources and troubleshooting
References
References provided by MGI
Selected Reference(s)
Raimundo N; Song L; Shutt TE; McKay SE; Cotney J; Guan MX; Gilliland TC; Hohuan D; Santos-Sacchi J; Shadel GS. 2012. Mitochondrial Stress Engages E2F1 Apoptotic Signaling to Cause Deafness. Cell 148(4):716-26. [PubMed: 22341444] [MGI Ref ID J:181548]
Health & husbandry
Health & Colony Maintenance Information
Animal Health Reports
Room Number FGB27
Colony Maintenance
Breeding & Husbandry When maintaining a live colony, hemizygous mice may be bred to wildtype (non-carrier) mice from the colony or to C57BL/6J inbred mice (Stock No. 000664). Mating System Noncarrier x Hemizygote (Female x Male) 28-AUG-12 Hemizygote x Noncarrier (Female x Male) 28-AUG-12 Diet Information LabDiet® 5K52/5K67
Pricing and Purchasing
Pricing, Supply Level & Notes, Controls
| Pricing for USA, Canada and Mexico shipping destinations |
|
Live Mice
Price per mouse (US dollars $) Gender Genotypes Provided Individual Mouse $232.00 Female or Male Hemizygous for Tg(CAG-Tfb1m)AGsha
Price per Pair (US dollars $) Pair Genotype $296.00 Hemizygous for Tg(CAG-Tfb1m)AGsha x Noncarrier $296.00 Noncarrier x Hemizygous for Tg(CAG-Tfb1m)AGsha Standard Supply
Repository-Live. Repository-Live represents an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. Repository-live orders are treated as custom orders. Within 2 business days, we respond to each availability inquiry or order with various delivery options. Repository Strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping.
| Pricing for International shipping destinations |
|
Live Mice
Price per mouse (US dollars $) Gender Genotypes Provided Individual Mouse $301.60 Female or Male Hemizygous for Tg(CAG-Tfb1m)AGsha
Price per Pair (US dollars $) Pair Genotype $384.80 Hemizygous for Tg(CAG-Tfb1m)AGsha x Noncarrier $384.80 Noncarrier x Hemizygous for Tg(CAG-Tfb1m)AGsha Standard Supply
Repository-Live. Repository-Live represents an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. Repository-live orders are treated as custom orders. Within 2 business days, we respond to each availability inquiry or order with various delivery options. Repository Strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping.
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Standard Supply
Repository-Live. Repository-Live represents an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. Repository-live orders are treated as custom orders. Within 2 business days, we respond to each availability inquiry or order with various delivery options. Repository Strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping.
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 000664 C57BL/6J | (approximate) | |
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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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 Information
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