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Former Names STOCK Hdtm2Mem/J (Changed: 13-DEC-07 ) B6.C-H61b Mod1a/(HW23)By (Changed: 19-SEP-07 ) Type Mutant Stock; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Generation F?+10p (30-MAY-04)
Generation DefinitionsDonating Investigator Dr. Marcy MacDonald, Massachusetts General Hospital Description
Mice with the Htttm2Mem targeted mutation display no overt phenotype. This strain carries 20 CAG repeat units in the first exon of the endogenous Hdh gene. This strain is a control for Stock No's. 003455 and 003456).Development
This strain carries 20 CAG repeat units in the first exon of the endogenous Htt gene.
| Control | ||
|---|---|---|
| None Available | ||
| Considerations for Choosing Controls | ||
Huntington's Disease Models
007708 B6.129-Gt(ROSA)26Sortm1(HD*103Q)Xwy/J 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 003455 STOCK Htttm4Mem/J 003456 STOCK Htttm5Mem/J View Huntington's Disease Models (28 strains)
Strains carrying other alleles of Htt
003454 B6.129-Htttm3Mem/J 003597 B6.129-Htttm4Mem/J 003598 B6.129-Htttm5Mem/J 016522 B6.129P2-Htttm2Detl/100J 004595 B6.129P2-Htttm2Detl/150J 016523 B6.129P2-Htttm2Detl/200J 016524 B6.129P2-Htttm2Detl/250J 016525 B6.129P2-Htttm2Detl/315J 021193 B6.129P2-Htttm2Detl/365J 016521 B6.129P2-Htttm2Detl/50J 002688 B6.129S4-Htttm1Mem/J 003455 STOCK Htttm4Mem/J 003456 STOCK Htttm5Mem/J View Strains carrying other alleles of Htt (13 strains)
Visit the Huntington's Diease Resource site for helpful information on Huntington's Disease and research resources.
View Related Disease (OMIM) Terms
Related Disease (OMIM) Terms provided by MGI
- No similarity to the expected human disease phenotype was found. One or more human genes are associated with this human disease. The mouse genotype may involve mutations to orthologs of one or more of these genes, but the phenotype did not resemble the disease.
Huntington Disease; HD
View Mammalian Phenotype Terms
Mammalian Phenotype Terms provided by MGI
assigned by genotype
Htttm2Mem/Htttm2Mem
involves: 129S1/Sv * 129X1/SvJ * CD-1
- nervous system phenotype
- *normal* nervous system phenotype
- unlike other Hdh alleles that are models for Huntington Disease, homozygotes show no change in striatal mitochondrial sensitivity to calcium in the brain, thus displaying normal striatal and cortical calcium sensitivity (MGI Ref ID J:99425)
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:Htttm2Mem related
Neurobiology Research
Cortical Defects
Huntington's disease
Neurodegeneration
| Allele Symbol | Htttm2Mem | ||
|---|---|---|---|
| Allele Name | targeted mutation 2, Marcy E MacDonald | ||
| Allele Type | Targeted (knock-in) | ||
| Common Name(s) | HdhQ20; | ||
| Mutation Made By | Dr. Marcy MacDonald, Massachusetts General Hospital | ||
| 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<+> | ||
| Promoter | Htt, huntingtin, mouse, laboratory | ||
| Molecular Note | This allele carries 18 CAG repeat units, with 20 glutamines, in the first exon of the endogenous gene, modeling the number of CAG repeats found in the human Hdh. It is a derivative of Hdhtm6Mem in which the neo cassette has been removed via cre-mediated recombination. [MGI Ref ID J:52440] | ||
Genotyping Protocols
Hdhtm2Mem, Separated PCR
Helpful Links
Genotyping resources and troubleshooting
White JK; Auerbach W; Duyao MP; Vonsattel JP; Gusella JF; Joyner AL ; MacDonald ME. 1997. Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion. Nat Genet 17(4):404-10. [PubMed: 9398841] [MGI Ref ID J:44391]
Htttm2Mem relatedAuerbach W; Hurlbert MS; Hilditch-Maguire P; Wadghiri YZ; Wheeler VC; Cohen SI; Joyner AL; MacDonald ME; Turnbull DH. 2001. The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin. Hum Mol Genet 10(22):2515-23. [PubMed: 11709539] [MGI Ref ID J:72915]
Brustovetsky N; LaFrance R; Purl KJ; Brustovetsky T; Keene CD; Low WC; Dubinsky JM. 2005. Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's Disease. J Neurochem 93(6):1361-70. [PubMed: 15935052] [MGI Ref ID J:99425]
Goula AV; Berquist BR; Wilson DM 3rd; Wheeler VC; Trottier Y; Merienne K. 2009. Stoichiometry of base excision repair proteins correlates with increased somatic CAG instability in striatum over cerebellum in Huntington's disease transgenic mice. PLoS Genet 5(12):e1000749. [PubMed: 19997493] [MGI Ref ID J:161747]
Jacobsen JC; Gregory GC; Woda JM; Thompson MN; Coser KR; Murthy V; Kohane IS; Gusella JF; Seong IS; Macdonald ME; Shioda T; Lee JM. 2011. HD CAG-correlated gene expression changes support a simple dominant gain of function. Hum Mol Genet 20(14):2846-60. [PubMed: 21536587] [MGI Ref ID J:173410]
Molero AE; Gokhan S; Gonzalez S; Feig JL; Alexandre LC; Mehler MF. 2009. Impairment of developmental stem cell-mediated striatal neurogenesis and pluripotency genes in a knock-in model of Huntington's disease. Proc Natl Acad Sci U S A :. [PubMed: 19955426] [MGI Ref ID J:155518]
Ribeiro FM; Paquet M; Ferreira LT; Cregan T; Swan P; Cregan SP; Ferguson SS. 2010. Metabotropic glutamate receptor-mediated cell signaling pathways are altered in a mouse model of Huntington's disease. J Neurosci 30(1):316-24. [PubMed: 20053912] [MGI Ref ID J:164039]
Sathasivam K; Neueder A; Gipson TA; Landles C; Benjamin AC; Bondulich MK; Smith DL; Faull RL; Roos RA; Howland D; Detloff PJ; Housman DE; Bates GP. 2013. Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease. Proc Natl Acad Sci U S A 110(6):2366-70. [PubMed: 23341618] [MGI Ref ID J:193824]
Snider BJ; Moss JL; Revilla FJ; Lee CS; Wheeler VC; Macdonald ME; Choi DW. 2003. Neocortical neurons cultured from mice with expanded CAG repeats in the huntingtin gene: unaltered vulnerability to excitotoxins and other insults. Neuroscience 120(3):617-25. [PubMed: 12895502] [MGI Ref ID J:128151]
Wheeler VC; Auerbach W; White JK; Srinidhi J; Auerbach A; Ryan A ; Duyao MP ; Vrbanac V ; Weaver M ; Gusella JF ; Joyner AL ; MacDonald ME. 1999. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Hum Mol Genet 8(1):115-22. [PubMed: 9887339] [MGI Ref ID J:52440]
Animal Health Reports
Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.Colony Maintenance
Diet Information LabDiet® 5K52/5K67
| Pricing for USA, Canada and Mexico shipping destinations |
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Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2450.00 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.
Embryos
Price (US dollars $) Frozen Embryo $1600.00 Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
- Cryopreserved Embryos
Available to most shipping destinations1
This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.
1 Shipments cannot be made to Australia due to Australian government import restrictions.
2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.Cryorecovery - Standard.
Progeny testing is not required.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. 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. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 11 and 14 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 |
|
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $3185.00 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.
Embryos
Price (US dollars $) Frozen Embryo $2080.00 Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
- Cryopreserved Embryos
Available to most shipping destinations1
This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.
1 Shipments cannot be made to Australia due to Australian government import restrictions.
2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.Cryorecovery - Standard.
Progeny testing is not required.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. 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. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 11 and 14 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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|
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
| Control | ||
|---|---|---|
| None Available | ||
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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