Strain Name:

B6.129-Htttm4Mem/J

Stock Number:

003597

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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 B6.129-Hdtm4Mem/J    (Changed: 13-DEC-07 )
B6.129-Hdhtm4Mem/J    (Changed: 19-SEP-07 )
Type Congenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain 129X1 x 129S1 via R1 (+Kitl-SlJ) ES cell line
GenerationN6F3p (30-MAY-04)
Generation Definitions
 
Donating Investigator IMR Colony,   The Jackson Laboratory

Description
This strain carries 92 CAG repeat units in the first exon of the endogenous Htt gene. Huntington's-like pathology is seen in the striatum, including nuclear localization of the protein, N-terminal inclusions, and insoluble aggregate formation. Instability of the length of the CAG repeat between generations is seen. Expression of this phenotype is delayed relative to Htttm5 (Stock No. 003456).

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. This is the case for the strain above. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available.

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

View Huntington's Disease Models     (22 strains)

Strains carrying   Htttm4Mem allele
003455   STOCK Htttm4Mem/J
View Strains carrying   Htttm4Mem     (1 strain)

Strains carrying other alleles of Htt
003454   B6.129-Htttm3Mem/J
003598   B6.129-Htttm5Mem/J
004595   B6.129P2-Htttm2Detl/J
002688   B6.129S4-Htttm1Mem/J
003453   STOCK Htttm2Mem/J
003455   STOCK Htttm4Mem/J
003456   STOCK Htttm5Mem/J
View Strains carrying other alleles of Htt     (7 strains)

Additional Web Information

Visit the Huntington's Diease Resource site for helpful information on Huntington's Disease and research resources.

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms
Huntington Disease; HD - Models with phenotypic similarity to human disease where etiologies involve orthologs.1
1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
View Mammalian Phenotype Terms

Mammalian Phenotype Terms
      assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Htttm4Mem/Htt+

        involves: 129S1/Sv * 129X1/SvJ * CD-1
  • nervous system phenotype
  • abnormal striatum morphology   (MGI Ref ID J:60937)
    • abnormal medium spiny neuron morphology
      • exhibit relocation of the mutant protein to the nucleus in medium sized spiny neurons and much later, the formation of morphologic nuclear inclusions and insoluble aggregate that are hallmarks of Huntington's Disease in humans, although at a slower rate than seen in homozygotes   (MGI Ref ID J:60937)
  • neuronal intranuclear inclusions   (MGI Ref ID J:60937)

Htttm4Mem/Htttm4Mem

        involves: 129S1/Sv * 129X1/SvJ * CD-1
  • nervous system phenotype
  • abnormal cerebral cortex morphology
    • QUIN and 3-HK levels are increased in the cortex beginning at 15 months of age, as seen in patients with Huntington disease   (MGI Ref ID J:111237)
  • abnormal nervous system physiology
    • between 5 and 12 months of age, striatal mitochondria develops resistance to calcium, becoming equally sensitive to calcium as cortical mitochondria, whereas in wild-type, striatal mitochondria is more sensitive to calcium than cortical mitochondria   (MGI Ref ID J:99425)
  • abnormal striatum morphology
    • the levels of both the endogenous excitotoxin quinolinic acid (QUIN) and its bioprecursor, 3-hydroxykynurenine (3-HK) are increased in the striatum beginning at 15 months of age, similarly to that seen in Huntington disease patients   (MGI Ref ID J:111237)
    • abnormal medium spiny neuron morphology
      • exhibit relocation of the mutant protein to the nucleus in medium sized spiny neurons and much later, the formation of morphologic nuclear inclusions (at 12-15 months) and insoluble aggregate that are hallmarks of Huntington's Disease in humans   (MGI Ref ID J:60937)
  • neuronal intranuclear inclusions
    • observed at 12-15 months   (MGI Ref ID J:60937)
  • behavior/neurological phenotype
  • limb grasping
    • observe clasping behavior is some mutants by one year of age   (MGI Ref ID J:99425)
View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

Htttm4Mem related

Mouse/Human Gene Homologs
Huntington's disease (chorea)

Neurobiology Research
Cortical Defects
Huntington's disease
Neurodegeneration

Genes & Alleles

Gene & Allele Information

 
Allele Symbol Htttm4Mem
Allele Name targeted mutation 4, Marcy E MacDonald
Allele Type Targeted (knock-in)
Common Name(s) HdhQ92; Hdhtm4Mem;
Mutation Made By 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<+>
Gene Symbol and Name Htt, huntingtin
Chromosome 5
Gene Common Name(s) AI256365; C430023I11Rik; HD; Hd; Hdh; Huntington disease (human); Huntington disease gene homolog; IT15; RIKEN cDNA C430023I11 gene; expressed sequence AI256365;
Molecular Note This allele carries 90 CAG repeat units, with 92 glutamines, in the first exon of the endogenous gene. [MGI Ref ID J:52440]

Genotyping

Genotyping Information

Genotyping Protocols

Htttm4Mem, Separated PCR

Helpful Links

Genotyping resources and troubleshooting

References

References

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

Wheeler VC; White JK; Gutekunst CA; Vrbanac V; Weaver M; Li XJ; Li SH; Yi H; Vonsattel JP; Gusella JF; Hersch S; Auerbach W; Joyner AL; MacDonald ME. 2000. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum Mol Genet 9(4):503-13. [PubMed: 10699173]  [MGI Ref ID J:60937]

Htttm4Mem related

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]

Cyr M; Sotnikova TD; Gainetdinov RR; Caron MG. 2006. Dopamine enhances motor and neuropathological consequences of polyglutamine expanded huntingtin. FASEB J 20(14):2541-3. [PubMed: 17065224]  [MGI Ref ID J:146707]

Fossale E; Wheeler VC; Vrbanac V; Lebel LA; Teed A; Mysore JS; Gusella JF; MacDonald ME; Persichetti F. 2002. Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice. Hum Mol Genet 11(19):2233-41. [PubMed: 12217951]  [MGI Ref ID J:79126]

Guidetti P; Bates GP; Graham RK; Hayden MR; Leavitt BR; MacDonald ME; Slow EJ; Wheeler VC; Woodman B; Schwarcz R. 2006. Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice. Neurobiol Dis 23(1):190-7. [PubMed: 16697652]  [MGI Ref ID J:111237]

Kuhn A; Goldstein DR; Hodges A; Strand AD; Sengstag T; Kooperberg C; Becanovic K; Pouladi MA; Sathasivam K; Cha JH; Hannan AJ; Hayden MR; Leavitt BR; Dunnett SB; Ferrante RJ; Albin R; Shelbourne P; Delorenzi M; Augood SJ; Faull RL; Olson JM; Bates GP; Jones L; Luthi-Carter R. 2007. Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum Mol Genet 16(15):1845-61. [PubMed: 17519223]  [MGI Ref ID J:142943]

Lynch G; Kramar EA; Rex CS; Jia Y; Chappas D; Gall CM; Simmons DA. 2007. Brain-derived neurotrophic factor restores synaptic plasticity in a knock-in mouse model of Huntington's disease J Neurosci 27(16):4424-4434. [PubMed: 17442827]  [MGI Ref ID J:119836]

Namura S; Hirt L; Wheeler VC; McGinnis KM; Hilditch-Maguire P; Moskowitz MA; MacDonald ME; Persichetti F. 2002. The HD mutation does not alter neuronal death in the striatum of Hdh(Q92) knock-in mice after mild focal ischemia. Neurobiol Dis 11(1):147-54. [PubMed: 12460554]  [MGI Ref ID J:125447]

Trueman RC; Brooks SP; Jones L; Dunnett SB. 2009. Rule learning, visuospatial function and motor performance in the Hdh(Q92) knock-in mouse model of Huntington's disease. Behav Brain Res 203(2):215-22. [PubMed: 19445966]  [MGI Ref ID J:150366]

Trueman RC; Brooks SP; Jones L; Dunnett SB. 2007. The operant serial implicit learning task reveals early onset motor learning deficits in the Hdh knock-in mouse model of Huntington's disease. Eur J Neurosci 25(2):551-8. [PubMed: 17284197]  [MGI Ref ID J:118627]

Trueman RC; Brooks SP; Jones L; Dunnett SB. 2008. Time course of choice reaction time deficits in the Hdh(Q92) knock-in mouse model of Huntington's disease in the operant Serial Implicit Learning Task (SILT). Behav Brain Res 189(2):317-24. [PubMed: 18367261]  [MGI Ref ID J:133206]

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]

Wheeler VC; White JK; Gutekunst CA; Vrbanac V; Weaver M; Li XJ; Li SH; Yi H; Vonsattel JP; Gusella JF; Hersch S; Auerbach W; Joyner AL; MacDonald ME. 2000. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum Mol Genet 9(4):503-13. [PubMed: 10699173]  [MGI Ref ID J:60937]

Health & husbandry

Health & Colony Maintenance Information

Currently there is no information available for this strain. This may be due to the supply level of this strain.

Purchasing information

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing
Order this mouse

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $1900.00
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.

Embryos

Price (US dollars $)
Frozen Embryo $1600.00

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.

Supply Notes

  • Cryopreserved Embryos
    This strain is also available as cryopreserved embryos. 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.
  • 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 View USA Canada and Mexico Pricing
Order this mouse

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2470.00
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.

Embryos

Price (US dollars $)
Frozen Embryo $2080.00

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.

Supply Notes

  • Cryopreserved Embryos
    This strain is also available as cryopreserved embryos. 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.
  • 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).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.

General Supply Notes

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
  International - 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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