Strain Name:

STOCK Htttm2Mem/J

Stock Number:

003453

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Cryopreserved - Ready for recovery

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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

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.
Specieslaboratory mouse
 
Donating InvestigatorDr. Marcy E 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 Information

  Control
   None Available
 
  Considerations for Choosing Controls

Related Strains

View Huntington's Disease Models     (29 strains)

View Strains carrying other alleles of Htt     (13 strains)

Additional Web Information

Visit our Huntington's Disease page for a full listing of Huntington's strains and research services.

Phenotype

Phenotype Information

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

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Htttm2Mem
Allele Name targeted mutation 2, Marcy E MacDonald
Allele Type Targeted
Common Name(s) HdhQ20;
Mutation Made ByDr. Marcy MacDonald,   Massachusetts General Hospital
Strain of Origin(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
ES Cell Line NameR1
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

Genotyping Information

Genotyping Protocols

Hdhtm2Mem, Separated PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

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]

Additional References

Htttm2Mem related

Auerbach 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]

Mielcarek M; Landles C; Weiss A; Bradaia A; Seredenina T; Inuabasi L; Osborne GF; Wadel K; Touller C; Butler R; Robertson J; Franklin SA; Smith DL; Park L; Marks PA; Wanker EE; Olson EN; Luthi-Carter R; van der Putten H; Beaumont V; Bates GP. 2013. HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration. PLoS Biol 11(11):e1001717. [PubMed: 24302884]  [MGI Ref ID J:205270]

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; Devries RA; Hamilton A; Guimaraes IM; Cregan SP; Pires RG; Ferguson SS. 2014. Metabotropic glutamate receptor 5 knockout promotes motor and biochemical alterations in a mouse model of Huntington's disease. Hum Mol Genet 23(8):2030-42. [PubMed: 24282028]  [MGI Ref ID J:207093]

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]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2525.00
Animals Provided

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.

Frozen Products

Price (US dollars $)
Frozen Embryo $1650.00

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 10 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 View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3283.00
Animals Provided

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.

Frozen Products

Price (US dollars $)
Frozen Embryo $2145.00

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 10 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).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

  Control
   None Available
 
  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.
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