Strain Name: |
B6.129-Gt(ROSA)26Sortm1(HD*103Q)Xwy/J |
|---|---|
Stock Number: |
007708 |
Availability: | Repository- Live |
General Terms and Conditions |
| Genes & Alleles | Gt(ROSA)26Sor; Gt(ROSA)26Sortm1(HD*103Q)Xwy; |
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 - Targeted Mutation Mating System Heterozygote x +/+ sibling (Female x Male) Species laboratory mouse Donating Investigator X. William Yang, University of California Los Angeles Generation N12+F1 (17-JUL-08) Strain Description
Mice heterozygous for the RosaHD mutant allele are viable and fertile. These mice have the neuropathogenic polyQ-mutant variant of the human Huntingtin protein (mhtt-exon1; 103Q) inserted into the Gt(ROSA)26Sor locus. Expression of mhtt-exon1 is blocked by an upstream loxP-flanked transcriptional STOP sequence. When bred to mice with a Cre recombinase gene under the control of a promoter of interest, the STOP sequence is deleted in the tissue of interest, and mhtt-exon1 expression is observed. As these RosaHD mutant mice allow cre-conditional expression of the neuropathogenic mhtt-exon1 protein, they may be useful in studying Huntington's disease (HD) or other polyQ disorders. Of note, sequencing of the polyQ region (using mice from the 11th backcross) indicate the actual number of repeats to be 98.For example, when bred to strains expressing cre in brain tissues (such as Nestin-Cre (see Stock No. 003771) or Emx1-Cre (see Stock No. 005628), bi-transgenic offspring show pathological cell-cell interactions critically contribute to cortical pathogenesis of HD.
Strain Development
A targeting vector was designed with a loxP-flanked transcriptional STOP sequence immediately upstream of a polyQ-mutant form of the human Huntingtin protein exon 1 (mhtt-exon1) sequence (containing 103 mixed CAA-CAG repeats, each encoding glutamine), all followed by a polyadenylation sequence. This targeting construct was incorporated into the Gt(ROSA)26Sor locus via electroporation into (129X1/SvJ x 129S1/Sv)F1-derived R1 embryonic stem (ES) cells. Correctly targeted ES cells were microinjected into C57BL/6J blastocysts. The resulting "RosaHD" mutant mice were subsequently backcrossed to C57BL/6J mice for at least 11 generations prior to arrival at The Jackson Laboratory. Of note, sequencing of the polyQ region (using mice from the 11th backcross) indicate the actual number of repeats to be 98.
Related Disease (OMIM) Terms |
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Gt(ROSA)26Sortm1(HD*103Q)Xwy | ||
|---|---|---|---|
| Allele Name | targeted mutation 1, X William Yang | ||
| Common Name(s) | RosaHD; mhtt-exon1 (103Q); | ||
| Strain of Origin | (129X1/SvJ x 129S1/Sv)F1-Kitl+ | ||
| ES Cell Line Name | R1 | ||
| ES Cell Line Strain | (129X1/SvJ x 129S1/Sv)F1-Kitl<+> | ||
| Gene Symbol and Name | Gt(ROSA)26Sor, gene trap ROSA 26, Philippe Soriano | ||
| Chromosome | 6 | ||
| Gene Common Name(s) | AV258896; Gtrgeo26; Gtrosa26; R26; ROSA26; beta geo; expressed sequence AV258896; gene trap ROSA 26; gene trap ROSA b-geo 26; | ||
| Molecular Note | A targeting vector was designed with a loxP-flanked transcriptional STOP sequence immediately upstream of a neuropathogenic polyQ-mutant form of the human Huntingtin protein exon 1 (mhtt-exon1) sequence (containing 103 mixed CAA-CAG repeats, each encoding glutamine), all followed by a polyadenylation sequence. When these mice are bred with animals expressing cre under the control of a promoter of interest, The STOP signal is deleted in tissue of interest allowing conditional expression of mhtt1-exon1. [MGI Ref ID J:123199] [MGI Ref ID J:99759] | ||
Control Information
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Genotyping Protocols
Gt(ROSA)26Sortm1(HD*103Q)Xwy
Colony Maintenance
| Breeding & Husbandry | When maintaining a live colony, heterozygous mice may be bred to wildtype siblings or to C57BL/6J inbred mice. While presumed to be viable and fertile, the phenotype of homozygous mice has not been reported (Aug-2007). |
|---|---|
| Diet Information | LabDiet® 5K52/5K67 |
Related Strains
Strains carrying other alleles of Gt(ROSA)26Sor
View Strains carrying other alleles of Gt(ROSA)26Sor (37 strains)
Additional Web Information
Congenic Nomenclature
Cre-lox or FLP-FRT Systems
Animal Health Reports
Room Number AX12
Research Applications
This mouse can be used to support research in many areas including:
Mouse/Human Gene Homologs
Huntington's disease (chorea)
Neurobiology Research
Behavioral and Learning Defects
Huntington's disease
Research Tools
Cre-lox System (loxP-flanked Sequences)
References
Selected Reference(s)
Additional ReferencesGu X; Li C; Wei W; Lo V; Gong S; Li SH; Iwasato T; Itohara S; Li XJ; Mody I; Heintz N; Yang XW. 2005. Pathological cell-cell interactions elicited by a neuropathogenic form of mutant Huntingtin contribute to cortical pathogenesis in HD mice. Neuron 46(3):433-44. [PubMed: 15882643] [MGI Ref ID J:99759]
Price and Supply Information
| Strain Name: | B6.129-Gt(ROSA)26Sortm1(HD*103Q)Xwy/J |
| Stock Number: | 007708 |
Price Details
IMPORTANT NOTE: Prices are based on shipping destination. To view prices, select your shipping destination.
*NO Shipping Destination selected!
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 for Licensing and Use Restrictions |
| Control Information | View Control Information in Strain Details. |
General Terms and Conditions
View JAX® Mice & Services Conditions of Use.
Effective September 26, 2007: License Requirements for Strains using Cre-lox Technology only apply in Canada, see Licenses for Strains using Cre-lox Technology.
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
Purchasing Information
JAX® Mice Orders
Surgical Services
Contact Information
Orders & Technical Support
Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form