Strain Name:

B6.129P2-Htttm2Detl/150J

Stock Number:

004595

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Availability:

Cryopreserved - Ready for recovery

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Use Restrictions Apply, see Terms of Use
These HdhQ150 mice carry a mutant knock in allele with a 150 CAG/polyQ repeat mutation. This mutant strain may be useful in studies related to Huntington's disease.

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 B6.129P2-Htttm2Detl/J    (Changed: 21-JUL-11 )
B6.129P2-Hdtm2Detl/J    (Changed: 13-DEC-07 )
B6.129P2-Hdhtm2Detl/J    (Changed: 19-SEP-07 )
STOCK Hdhtm2Detl/J    (Changed: 15-DEC-04 )
Type Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
 
Donating Investigator Gillian P Bates,   United Medical and Dental Schools

Description
Mice homozygous for the targeted allele are viable and fertile. At 15-40 weeks of age mice carrying this allele on a segregating C57BL/6 and 129P2 background exhibit an abnormal gait, clasping behavior and diminished exploratory activity. Infrequent tonic-clonic like seizures may also be observed. Mice with a higher percentage of C57BL/6 in their genetic background develop behavioral and neurological phenotypes at a much later age (70-100 weeks). (Heng MY et al. 2007) Mutant mice may be noticeably smaller than wild-type littermates. Increased glial fibrillary acidic protein immunoreactivity is present in the striatum and ubiquititin- and huntingtin-positive neuronal intranuclear inclusions (NIIs) are detected throughout the dorsal striatum, nucleus accumbens and to a lesser extent other regions of the brain. Onset of symptoms occurs earlier for homozygotes than for heterozygotes.This mutant mouse strain represents a model that may be useful in studies related to Huntington's disease.

The Repository distributes a set of strains containing different CAG/polyQ repeat length mutants derived from this HdhQ150 strain. The set includes, STOCK numbers: 016521, 016522, 016523, 016524, 016525.

Development
In the first step of a two step procedure, exon 1 was replaced with the selectable marker Hprt in 129P2/OlaHsd-derived HM1 embryonic stem (ES) cells. A second round of targeting was performed to replace Hprt with exon 1 sequence including a 150 CAG repeat segment. Correctly targeted ES cells were injected into C57BL/6J blastocysts. The mice were backcrossed to C57BL/6J for at least eight generations(12/20/02) before being donated to the Repository. Upon arrival at The Jackson Laboratory, the mice were crossed to C57BL/6J (Stock No. 000664) at least once to establish the colony.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

View Huntington's Disease Models     (29 strains)

Strains carrying   Htttm2Detl allele
016522   B6.129P2-Htttm2Detl/100J
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
View Strains carrying   Htttm2Detl     (6 strains)

Strains carrying other alleles of Htt
003454   B6.129-Htttm3Mem/J
003597   B6.129-Htttm4Mem/J
003598   B6.129-Htttm5Mem/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 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
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Huntington Disease; HD
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Htttm2Detl/Htt+

        involves: 129P2/OlaHsd * C57BL/6J
  • mortality/aging
  • premature death
    • only 3 of 45 heterozygotes die prior to 1 year of age; heterozygotes typically survive for >1 year   (MGI Ref ID J:67074)
  • behavior/neurological phenotype
  • abnormal behavior
    • the discrepancy between onset age of behavioral and neurological phenotypes is believed to reflect the percentage of C57BL/6 in the strain background (50-75% C57BL/6 - early onset (J:67074), 75-90% C57BL/6 - late onset (J:123681))   (MGI Ref ID J:123681)
    • abnormal motor capabilities/coordination/movement
      • heterozygotes are viable and developmentally normal but present symptoms of motor deficits with an onset of ~60 weeks; symptom presentation is variable among individual mutants   (MGI Ref ID J:67074)
      • abnormal gait
        • old heterozygotes (greater than 40 weeks) exhibit an abnormal gait relative to wild-type mice   (MGI Ref ID J:67074)
        • old (but not young) heterozygotes display a 2-fold increase in stride and base length variation relative to wild-type mice, suggesting that the mutation causes a late-onset variability in gait   (MGI Ref ID J:67074)
        • staggering gait observed in animals at 100 weeks of age   (MGI Ref ID J:123681)
        • short stride length
          • hindpaw and forepaw stride lengths are reduced in heterozygotes at 100 weeks of age as compared to wild-type   (MGI Ref ID J:123681)
      • abnormal motor coordination/ balance
        • at 5.0 rpm., heterozygotes (15-40 weeks) stay for a significantly shorter time on a slowly rotating rod relative to age-matched wild-type mice   (MGI Ref ID J:67074)
        • enhanced coordination
          • at 20 weeks of age mice performed better in motor performance on the accelerated rotarod than wild-type controls   (MGI Ref ID J:123681)
          • heterozygotes outperform homozygotes on the rotarod up to 40 weeks of age   (MGI Ref ID J:123681)
        • impaired balance
          • beginning at 70 weeks of age time to traverse 11 mm round and 5 mm square balance beam is increased as compared to wild-type   (MGI Ref ID J:123681)
          • 50% of 100 week old mice fail to traverse the 5 mm beam   (MGI Ref ID J:123681)
          • 63% of 100 week old mice exhibit a hindlimb drag while traversing beam   (MGI Ref ID J:123681)
        • impaired limb coordination
          • at >40 weeks of age, heterozygotes show a late-onset increase in the average distance between front and hind paws ('overlap' distance) relative to age-matched wild-type mice or Hdhtm1Detl heterozygotes (carrying a 80 unit CAG repeat)   (MGI Ref ID J:67074)
      • hypoactivity
        • at 15-40 weeks of age, a few heterozygotes remain inactive upon removal of the cage lid whereas all wild-type mice show exploratory activity by walking around the cage   (MGI Ref ID J:67074)
        • the proportion of heterozygotes tending to remain inactive increases significantly with age (>40 weeks), indicating that the tendency to be inactive is a late-onset trait conferred by the mutant allele   (MGI Ref ID J:67074)
      • limb grasping
        • during tail suspension, >60% of old heterozygotes (>40 weeks) versus only <20% of wild-type or Hdhtm1Detl homozygotes (carrying a 80 unit CAG repeat) tend to clasp   (MGI Ref ID J:67074)
        • the tendency to clasp is a late-onset trait conferred by the Hdhtm2Detl allele   (MGI Ref ID J:67074)
  • nervous system phenotype
  • *normal* nervous system phenotype
    • heterozygotes display no extreme reductions in major brain regions up to 52 weeks of age   (MGI Ref ID J:67074)
    • abnormal dorsal striatum morphology
      • striatal dopamine D1 receptor sites are decreased by 42% as compared to wild-type at 100 weeks of age   (MGI Ref ID J:123681)
      • striatal dopamine D2 receptor sites are decreased by 17% as compared to wild-type at 100 weeks of age   (MGI Ref ID J:123681)
    • abnormal ventral striatum morphology
      • striatal dopamine D1 receptor sites are decreased by 50% as compared to wild-type at 100 weeks of ag   (MGI Ref ID J:123681)
      • striatal dopamine D2 receptor sites are decreased by 17% as compared to wild-type at 100 weeks of age   (MGI Ref ID J:123681)
    • gliosis
      • mutant mice exhibit significant reactive gliosis in the striatum; notably, neuronal number remains relatively unaffected   (MGI Ref ID J:67074)
    • neuron degeneration
      • starting at ~40 weeks, mutants exhibit neuronal intranuclear inclusions (NIIs) predominantly in the striatum; however, no dystrophic neurites are observed   (MGI Ref ID J:67074)
  • growth/size/body phenotype
  • decreased body size
    • at 52 weeks of age, some heterozygotes are significantly smaller than wild-type littermates   (MGI Ref ID J:67074)
  • homeostasis/metabolism phenotype
  • *normal* homeostasis/metabolism phenotype
    • heterozygotes display normal blood glucose levels relative to wild-type mice   (MGI Ref ID J:67074)

Htttm2Detl/Htttm2Detl

        involves: 129P2/OlaHsd * C57BL/6J
  • behavior/neurological phenotype
  • abnormal behavior
    • the discrepancy between onset age of behavioral and neurological phenotypes is believed to reflect the percentage of C57BL/6 in the strain background (50-75% C57BL/6 - early onset (J:67074), 75-90% C57BL/6 - late onset (J:123681))   (MGI Ref ID J:123681)
    • abnormal motor capabilities/coordination/movement
      • homozygotes are viable and developmentally normal but present symptoms of motor deficits with an onset of ~25 weeks; symptom presentation is variable among individual mutants   (MGI Ref ID J:67074)
      • at 20 weeks of age mice performed better in motor learning and motor performance on the accelerated rotarod than wild-type controls   (MGI Ref ID J:123681)
      • muscle power as assessed by latency to fall in the hanging wire test is not impaired in 100 week old mice   (MGI Ref ID J:123681)
      • abnormal gait
        • young homozygotes (15-40 weeks) exhibit a more severe gait abnormality than age-matched heterozygotes   (MGI Ref ID J:67074)
        • 9 out of 10 homozygotes with severe gait disturbance display either clasping or open cage inactivity   (MGI Ref ID J:67074)
        • staggering gait/loss of gait pattern is observed in homozygotes at 100 weeks of age   (MGI Ref ID J:123681)
        • short stride length
          • hindpaw and forepaw stride lengths are reduced in homozygotes at 100 weeks of age as compared to wild-type   (MGI Ref ID J:123681)
      • abnormal motor coordination/ balance
        • at 5.0 rpm., homozygotes (15-40 weeks) stay for a significantly shorter time on a slowly rotating rod than age-matched heterozygotes   (MGI Ref ID J:67074)
        • impaired balance
          • beginning at 70 weeks of age time to traverse 11 mm round and 5 mm square balance beams is increased as compared to wild-type   (MGI Ref ID J:123681)
          • 80% of 100 week old mice fail to traverse the 5 mm beam   (MGI Ref ID J:123681)
          • as early as 40 weeks of age, homozygotes exhibit a hindlimb drag while traversing beam   (MGI Ref ID J:123681)
          • 90% of 100 week old mice exhibit a hindlimb drag while traversing beam   (MGI Ref ID J:123681)
        • impaired coordination
          • significant increase in number of falls on the accelerated rotarod at 100 weeks of age in comparison to wild-type and heterozygotes, however, motor learning is not impaired   (MGI Ref ID J:123681)
        • impaired limb coordination
          • at >40 weeks of age, homozygotes exhibit an early-onset increase in the distance between front and hind paws ('overlap' distance) relative to age-matched heterozygotes   (MGI Ref ID J:67074)
      • hypoactivity
        • homozygotes younger than 40 weeks of age tend to exhibit open cage inactivity, suggesting that an increase in mutant allele dose causes an earlier onset of this abnormality   (MGI Ref ID J:67074)
        • reduced activity first observed in animals at 70 weeks of age as measured by automated activity cages, by 100 weeks activity is significantly reduced   (MGI Ref ID J:123681)
      • limb grasping
        • during tail suspension, >60% of young homozygotes (15-40 weeks) versus only <20% of wild-type or Hdhtm1Detl homozygotes (carrying a 80 unit CAG repeat) tend to clasp   (MGI Ref ID J:67074)
        • homozygotes with a 150 unit CAG repeat exhibit an earlier onset of limb clasping relative to heterozygotes   (MGI Ref ID J:67074)
        • clasping behavior produced by tail suspension test is observed in a small number of homozygotes at 20 weeks of age; by 70 - 100 weeks of age clasping behavior is common   (MGI Ref ID J:123681)
      • tremors
        • resting tremor observed in animals at 100 weeks of age   (MGI Ref ID J:123681)
    • tonic-clonic seizures
      • 4 out of 25 mutant mice (250 trials; 25-60 weeks of age) exhibit a convulsive spell consistent with a tonic-clonic seizure; more than one occurrences are noted in two mice during a 10 trial period   (MGI Ref ID J:67074)
      • seizures are not observed in mice with higher percentage of C57BL/6 in genetic background   (MGI Ref ID J:123681)
  • growth/size/body phenotype
  • decreased body size
    • at >25 weeks of age, ~1 in 10 mutants appears significantly smaller than its wild-type littermate; this size difference progresses slowly with age   (MGI Ref ID J:67074)
    • weight loss
      • progressive weight loss with age   (MGI Ref ID J:123681)
      • most significant loss observed between 70 and 100 weeks of age as compared to wild-type   (MGI Ref ID J:123681)
  • homeostasis/metabolism phenotype
  • *normal* homeostasis/metabolism phenotype
    • homozygotes display normal blood glucose levels relative to wild-type mice   (MGI Ref ID J:67074)
  • nervous system phenotype
  • *normal* nervous system phenotype
    • homozygotes display no extreme reductions in major brain regions up to 52 weeks of age   (MGI Ref ID J:67074)
    • abnormal neuron morphology
      • striatal neurons appear atrophic and irregularly shaped   (MGI Ref ID J:123681)
      • neuron degeneration
        • starting at ~40 weeks, mutants exhibit neuronal intranuclear inclusions (NIIs) predominantly in the striatum; no dystrophic neurites are observed   (MGI Ref ID J:67074)
      • neuronal intranuclear inclusions
        • starting at ~40 weeks, mutants exhibit neuronal intranuclear inclusions (NIIs) predominantly in the striatum   (MGI Ref ID J:67074)
    • abnormal striatum morphology
      • homozygotes exhibit a 42.8% reduction in striatal neuron number   (MGI Ref ID J:123681)
      • mean striatal volume is reduced to 40.4% as compared to wild-type at 100 weeks of age   (MGI Ref ID J:123681)
      • abnormal dorsal striatum morphology
        • striatal dopamine D1 receptor sites are decreased by 79% as compared to wild-type at 100 weeks of age   (MGI Ref ID J:123681)
        • striatal dopamine D2 receptor sites are decreased by 42% as compared to wild-type at 100 weeks of age   (MGI Ref ID J:123681)
      • abnormal ventral striatum morphology
        • striatal dopamine D1 receptor sites are decreased by 86% as compared to wild-type at 100 weeks of age   (MGI Ref ID J:123681)
        • striatal dopamine D2 receptor sites are decreased by 32% as compared to wild-type at 100 weeks of age   (MGI Ref ID J:123681)
    • gliosis
      • mutant mice exhibit significant reactive gliosis in the striatum; notably, neuronal number remains relatively unaffected   (MGI Ref ID J:67074)
    • tonic-clonic seizures
      • 4 out of 25 mutant mice (250 trials; 25-60 weeks of age) exhibit a convulsive spell consistent with a tonic-clonic seizure; more than one occurrences are noted in two mice during a 10 trial period   (MGI Ref ID J:67074)
      • seizures are not observed in mice with higher percentage of C57BL/6 in genetic background   (MGI Ref ID J:123681)
View Research Applications

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

Htttm2Detl related

Neurobiology Research
Cortical Defects
Huntington's disease
Neurodegeneration

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Htttm2Detl
Allele Name targeted mutation 2, Peter J Detloff
Allele Type Targeted
Common Name(s) CHL2; Hdh150Q; Hdh200Q; Hdh(CAG)150; HdhQ150; HdhCAG150; HdhQ150;
Strain of Origin129P2/OlaHsd-Hprt
ES Cell Line NameHM-1
ES Cell Line Strain129P2/OlaHsd-Hprt
Promoter Htt, huntingtin, mouse, laboratory
Molecular Note This allele carries 150 CAG repeat units in the first exon of the endogenous gene. CAG repeat numbers in this gene are highly unstable. Mice carrying this allele may have more or fewer repeats than stated in the original description. [MGI Ref ID J:67074]

Genotyping

Genotyping Information

Genotyping Protocols

Htttm2Detl repeat assay, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Heng MY; Tallaksen-Greene SJ; Detloff PJ; Albin RL. 2007. Longitudinal evaluation of the Hdh(CAG)150 knock-in murine model of Huntington's disease. J Neurosci 27(34):8989-98. [PubMed: 17715336]  [MGI Ref ID J:123681]

Lin CH; Tallaksen-Greene S; Chien WM; Cearley JA; Jackson WS; Crouse AB; Ren S; Li XJ; Albin RL; Detloff PJ. 2001. Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum Mol Genet 10(2):137-44. [PubMed: 11152661]  [MGI Ref ID J:67074]

Additional References

Htttm2Detl related

Bjorkqvist M; Wild EJ; Thiele J; Silvestroni A; Andre R; Lahiri N; Raibon E; Lee RV; Benn CL; Soulet D; Magnusson A; Woodman B; Landles C; Pouladi MA; Hayden MR; Khalili-Shirazi A; Lowdell MW; Brundin P; Bates GP; Leavitt BR; Moller T; Tabrizi SJ. 2008. A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med 205(8):1869-77. [PubMed: 18625748]  [MGI Ref ID J:138558]

Bradford J; Shin JY; Roberts M; Wang CE; Li XJ; Li S. 2009. Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms. Proc Natl Acad Sci U S A 106(52):22480-5. [PubMed: 20018729]  [MGI Ref ID J:156460]

Brooks SP; Betteridge H; Trueman RC; Jones L; Dunnett SB. 2006. Selective extra-dimensional set shifting deficit in a knock-in mouse model of Huntington's disease. Brain Res Bull 69(4):452-7. [PubMed: 16624677]  [MGI Ref ID J:112811]

Chiang MC; Chen HM; Lee YH; Chang HH; Wu YC; Soong BW; Chen CM; Wu YR; Liu CS; Niu DM; Wu JY; Chen YT; Chern Y. 2007. Dysregulation of C/EBPalpha by mutant Huntingtin causes the urea cycle deficiency in Huntington's disease. Hum Mol Genet 16(5):483-98. [PubMed: 17213233]  [MGI Ref ID J:121854]

Crook ZR; Housman D. 2011. Huntington's disease: can mice lead the way to treatment? Neuron 69(3):423-35. [PubMed: 21315254]  [MGI Ref ID J:174750]

Dixon KT; Cearley JA; Hunter JM; Detloff PJ. 2004. Mouse Huntington's disease homolog mRNA levels: variation and allele effects. Gene Expr 11(5-6):221-31. [PubMed: 15200234]  [MGI Ref ID J:90356]

Dougherty SE; Reeves JL; Lesort M; Detloff PJ; Cowell RM. 2013. Purkinje cell dysfunction and loss in a knock-in mouse model of Huntington disease. Exp Neurol 240:96-102. [PubMed: 23195593]  [MGI Ref ID J:196980]

Gonitel R; Moffitt H; Sathasivam K; Woodman B; Detloff PJ; Faull RL; Bates GP. 2008. DNA instability in postmitotic neurons. Proc Natl Acad Sci U S A 105(9):3467-72. [PubMed: 18299573]  [MGI Ref ID J:132766]

Heng MY; Detloff PJ; Wang PL; Tsien JZ; Albin RL. 2009. In vivo evidence for NMDA receptor-mediated excitotoxicity in a murine genetic model of Huntington disease. J Neurosci 29(10):3200-5. [PubMed: 19279257]  [MGI Ref ID J:147069]

Heng MY; Duong DK; Albin RL; Tallaksen-Greene SJ; Hunter JM; Lesort MJ; Osmand A; Paulson HL; Detloff PJ. 2010. Early autophagic response in a novel knock-in model of Huntington disease. Hum Mol Genet 19(19):3702-20. [PubMed: 20616151]  [MGI Ref ID J:163641]

Her LS; Goldstein LS. 2008. Enhanced sensitivity of striatal neurons to axonal transport defects induced by mutant huntingtin. J Neurosci 28(50):13662-72. [PubMed: 19074039]  [MGI Ref ID J:143045]

Hsiao HY; Chen YC; Chen HM; Tu PH; Chern Y. 2013. A critical role of astrocyte-mediated nuclear factor-kappaB-dependent inflammation in Huntington's disease. Hum Mol Genet 22(9):1826-42. [PubMed: 23372043]  [MGI Ref ID J:194975]

Kennedy L; Evans E; Chen CM; Craven L; Detloff PJ; Ennis M; Shelbourne PF. 2003. Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis. Hum Mol Genet 12(24):3359-67. [PubMed: 14570710]  [MGI Ref ID J:136414]

Kim YJ; Sapp E; Cuiffo BG; Sobin L; Yoder J; Kegel KB; Qin ZH; Detloff P; Aronin N; DiFiglia M. 2006. Lysosomal proteases are involved in generation of N-terminal huntingtin fragments. Neurobiol Dis 22(2):346-56. [PubMed: 16423528]  [MGI Ref ID J:111314]

Labbadia J; Cunliffe H; Weiss A; Katsyuba E; Sathasivam K; Seredenina T; Woodman B; Moussaoui S; Frentzel S; Luthi-Carter R; Paganetti P; Bates GP. 2011. Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease. J Clin Invest 121(8):3306-19. [PubMed: 21785217]  [MGI Ref ID J:176008]

Li X; Wang CE; Huang S; Xu X; Li XJ; Li H; Li S. 2010. Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments. Hum Mol Genet 19(12):2445-55. [PubMed: 20354076]  [MGI Ref ID J:160460]

Lin YS; Chen CM; Soong BW; Wu YR; Chen HM; Yeh WY; Wu DR; Lin YJ; Poon PW; Cheng ML; Wang CH; Chern Y. 2011. Dysregulated brain creatine kinase is associated with hearing impairment in mouse models of Huntington disease. J Clin Invest 121(4):1519-23. [PubMed: 21403395]  [MGI Ref ID J:172008]

Lorincz MT; Zawistowski VA. 2009. Expanded CAG repeats in the murine Huntington's disease gene increases neuronal differentiation of embryonic and neural stem cells. Mol Cell Neurosci 40(1):1-13. [PubMed: 18625318]  [MGI Ref ID J:147008]

Marcellin D; Abramowski D; Young D; Richter J; Weiss A; Marcel A; Maassen J; Kauffmann M; Bibel M; Shimshek DR; Faull RL; Bates GP; Kuhn RR; Van der Putten PH; Schmid P; Lotz GP. 2012. Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon aging. PLoS One 7(9):e44457. [PubMed: 22984513]  [MGI Ref ID J:192895]

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]

Moffitt H; McPhail GD; Woodman B; Hobbs C; Bates GP. 2009. Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease. PLoS One 4(11):e8025. [PubMed: 19956633]  [MGI Ref ID J:155387]

Oliveira JM; Jekabsons MB; Chen S; Lin A; Rego AC; Goncalves J; Ellerby LM; Nicholls DG. 2007. Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice. J Neurochem 101(1):241-9. [PubMed: 17394466]  [MGI Ref ID J:144268]

Orr AL; Li S; Wang CE; Li H; Wang J; Rong J; Xu X; Mastroberardino PG; Greenamyre JT; Li XJ. 2008. N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking. J Neurosci 28(11):2783-92. [PubMed: 18337408]  [MGI Ref ID J:133021]

Proenca CC; Stoehr N; Bernhard M; Seger S; Genoud C; Roscic A; Paganetti P; Liu S; Murphy LO; Kuhn R; Bouwmeester T; Galimberti I. 2013. Atg4b-dependent autophagic flux alleviates huntington's disease progression. PLoS One 8(7):e68357. [PubMed: 23861892]  [MGI Ref ID J:204285]

Sathasivam K; Lane A; Legleiter J; Warley A; Woodman B; Finkbeiner S; Paganetti P; Muchowski PJ; Wilson S; Bates GP. 2010. Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease. Hum Mol Genet 19(1):65-78. [PubMed: 19825844]  [MGI Ref ID J:155118]

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]

Switonski PM; Fiszer A; Kazmierska K; Kurpisz M; Krzyzosiak WJ; Figiel M. 2011. Mouse ataxin-3 functional knock-out model. Neuromolecular Med 13(1):54-65. [PubMed: 20945165]  [MGI Ref ID J:175960]

Tallaksen-Greene SJ; Crouse AB; Hunter JM; Detloff PJ; Albin RL. 2005. Neuronal intranuclear inclusions and neuropil aggregates in Hdh(CAG(150)) knockin mice. Neuroscience 131(4):843-52. [PubMed: 15749339]  [MGI Ref ID J:96770]

Trushina E; Canaria CA; Lee DY; McMurray CT. 2014. Loss of caveolin-1 expression in knock-in mouse model of Huntington's disease suppresses pathophysiology in vivo. Hum Mol Genet 23(1):129-44. [PubMed: 24021477]  [MGI Ref ID J:203116]

Trushina E; Du Charme J; Parisi J; McMurray CT. 2006. Neurological abnormalities in caveolin-1 knock out mice. Behav Brain Res 172(1):24-32. [PubMed: 16750274]  [MGI Ref ID J:110712]

Wang CE; Tydlacka S; Orr AL; Yang SH; Graham RK; Hayden MR; Li S; Chan AW; Li XJ. 2008. Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. Hum Mol Genet 17(17):2738-51. [PubMed: 18558632]  [MGI Ref ID J:138148]

Woodman B; Butler R; Landles C; Lupton MK; Tse J; Hockly E; Moffitt H; Sathasivam K; Bates GP. 2007. The Hdh(Q150/Q150) knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes. Brain Res Bull 72(2-3):83-97. [PubMed: 17352931]  [MGI Ref ID J:128653]

Xun Z; Rivera-Sanchez S; Ayala-Pena S; Lim J; Budworth H; Skoda EM; Robbins PD; Niedernhofer LJ; Wipf P; McMurray CT. 2012. Targeting of XJB-5-131 to mitochondria suppresses oxidative DNA damage and motor decline in a mouse model of Huntington's disease. Cell Rep 2(5):1137-42. [PubMed: 23122961]  [MGI Ref ID J:196344]

Young D; Mayer F; Vidotto N; Schweizer T; Berth R; Abramowski D; Shimshek DR; van der Putten PH; Schmid P. 2013. Mutant huntingtin gene-dose impacts on aggregate deposition, DARPP32 expression and neuroinflammation in HdhQ150 mice. PLoS One 8(9):e75108. [PubMed: 24086450]  [MGI Ref ID J:206483]

Yu D; Pendergraff H; Liu J; Kordasiewicz HB; Cleveland DW; Swayze EE; Lima WF; Crooke ST; Prakash TP; Corey DR. 2012. Single-Stranded RNAs Use RNAi to Potently and Allele-Selectively Inhibit Mutant Huntingtin Expression. Cell 150(5):895-908. [PubMed: 22939619]  [MGI Ref ID J:187977]

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, G200.

Colony Maintenance

Breeding & HusbandryThis strain originated on a B6;129P2 background. When maintaining a live colony, these mice can be bred as heterozygotes. Onset of symptoms occurs earlier for homozygotes than for heterozygotes. Expected coat color is black.

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
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


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 Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


For Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license prior to shipping.

Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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