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| These RosaHD mutant mice allow cre-conditional expression of the neuropathogenic mhtt-exon1 protein and may be useful in studying Huntington’s disease or other polyQ disorders. | |||||||||||||||||||
- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Strain Information
Type Congenic; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Donating Investigator X. William Yang, University of California Los Angeles 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.
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.
Control Information
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Related Strains
Huntington's Disease Models
003454 B6.129-Htttm3Mem/J 003597 B6.129-Htttm4Mem/J 003598 B6.129-Htttm5Mem/J 016094 B6.129P2-Git2Gt(XG510)Byg/WeisJ 016522 B6.129P2-Htttm2Detl/100J 004595 B6.129P2-Htttm2Detl/150J 016521 B6.129P2-Htttm2Detl/50J 002688 B6.129S4-Htttm1Mem/J 006471 B6.Cg-Tg(HDexon1)61Gpb/J 008333 B6;129P2-Dldtm1Ptl/J 004360 B6;SJL-Tg(HD)63Aron/J 003627 B6C3-Tg(HD82Gln)81Dbo/J 002809 B6CBA-Tg(HDexon1)61Gpb/1J 002810 B6CBA-Tg(HDexon1)62Gpb/1J 006494 B6CBA-Tg(HDexon1)62Gpb/3J 016095 C.129P2(B6)-Git2Gt(XG510)Byg/WeisJ 008833 C57BL/6-Tg(Camk2a-UBB)3413-1Fwvl/J 007578 CBy.Cg-Tg(HDexon1)61Gpb/J 013732 FVB-Tg(NPEPPS)1Skar/J 004938 FVB-Tg(YAC128)53Hay/J 012630 FVB/N-Tg(GFAP-HTT*160Q)31Xjl/J 008197 FVB/N-Tg(HTT*97Q)IXwy/J 007247 FVB/N-Tg(YAC353G6)W7Hay/J 003640 FVB/NJ-Tg(YAC72)2511Hay/J 007749 STOCK Hap1tm1Xjl/J 003453 STOCK Htttm2Mem/J 003455 STOCK Htttm4Mem/J 003456 STOCK Htttm5Mem/J View Huntington's Disease Models (28 strains)
Additional Web Information
Introduction to Cre-lox technology
Visit the Huntington's Diease Resource site for helpful information on Huntington's Disease and research resources.
Phenotype
Phenotype Information
Huntington Disease; HD - Models with phenotypic similarity to human disease where etiologies are distinct.2
2 Human genes are associated with this disease. Orthologs of those genes do not appear in the mouse genotype(s). assigned by genotype
The following phenotype relates to a compound genotype created using this strain.
Contact JAX® Services jaxservices@jax.org for customized breeding options.Gt(ROSA)26Sortm1(HD*103Q)Xwy/? Tg(Nes-cre)1Kln/?
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL (conditional)
- nervous system phenotype
- abnormal cerebral cortex pyramidal cell morphology
- 35 of 37 neurons display dysmorphic axons compared to only 5 of 40 wild-type axons in cortex in 6-month old mice (MGI Ref ID J:99759)
- cerebral cortex pyramidal cell degeneration
- at 1 year of age, an increase in degenerating neurons, including pyramidal neurons, is seen in the cortex compared to wild-type mice (MGI Ref ID J:99759)
- abnormal inhibitory postsynaptic currents
- spontaneous inhibitory postsynaptic current frequency but not amplitude is significantly decreased; however no change in spontaneous excitatory postsynaptic currents is seen (MGI Ref ID J:99759)
- gliosis
- the density of GFAP+ cells is increased 9-fold and 6-fold in the cortex and striatum, respectively, compared to wild-type littermates at 6 months (MGI Ref ID J:99759)
- neurodegeneration
- at 1 year of age, an increase in degenerating neurons, including pyramidal neurons, is seen in the cortex compared to wild-type mice (MGI Ref ID J:99759)
- behavior/neurological phenotype
- hypoactivity
- activity is reduced at 6, 8, and 10 months of age but not at 2 months of age (MGI Ref ID J:99759)
This mouse can be used to support research in many areas including:
Mouse/Human Gene Homologs
Huntington's disease (chorea)
Neurobiology Research
Ataxia (Movement) Defects
Behavioral and Learning Defects
Huntington's disease
Neuromuscular Defects
Research Tools
Cre-lox System
loxP-flanked Sequences
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | Gt(ROSA)26Sortm1(HD*103Q)Xwy | ||
|---|---|---|---|
| Allele Name | targeted mutation 1, X William Yang | ||
| Allele Type | Targeted (knock-in) | ||
| 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] | ||
Genotyping
Genotyping Information
Genotyping Protocols
HD*103Q, Standard PCR
Gt(ROSA)26Sortm1(HD*103Q)Xwy, Standard PCR
Helpful Links
Genotyping resources and troubleshooting
References
References provided by MGI
Selected Reference(s)
Gu 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]
Additional References
Gt(ROSA)26Sortm1(HD*103Q)Xwy relatedKazantsev A; Preisinger E; Dranovsky A; Goldgaber D; Housman D. 1999. Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells. Proc Natl Acad Sci U S A 96(20):11404-9. [PubMed: 10500189] [MGI Ref ID J:123199]
Health & husbandry
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Health & Colony Maintenance Information
Animal Health Reports
Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, RG10/RG30.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).
Purchasing information
Pricing, Supply Level & Notes, Controls
| Pricing for USA, Canada and Mexico shipping destinations |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $1980.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
Supply Notes
- Cryorecovery - Standard.
We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 13 and 16 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
| Pricing for International shipping destinations |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2574.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
Supply Notes
- Cryorecovery - Standard.
We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 13 and 16 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
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Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
General Supply Notes
- This strain is included in the Induced Mutant Resource Colony collection.
Control Information
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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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 Information
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