Strain Name:

CBy.Cg-Tg(HDexon1)61Gpb/J

Stock Number:

007578

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Availability:

Cryopreserved - Ready for recovery

Use Restrictions Apply, see Terms of Use
These HDexon1 mice may be useful in Huntington's Disease research.

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; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
GenerationN26+pN1
Generation Definitions
 
Donating Investigator Gillian P Bates,   United Medical and Dental Schools

Description
Mice have been generated that are transgenic for the 5' end of the human HD gene carrying approximately 100 CAG repeat expansions. In this founder line (61Gpb), the transgene is ubiquitously expressed. Transgenic mice exhibit a progressive neurological phenotype that mimics many of the features of HD, including choreiform-like movements, involuntary stereotypic movements, tremor, and epileptic seizures, as well as nonmovement disorder components, including unusual vocalization. They urinate frequently and exhibit loss of body weight and muscle bulk through the course of the disease. Neurologically they develop Neuronal Intranuclear Inclusions (NII) which contain both the huntingtin and ubiquitin proteins. These NII have also been identified in human HD patients. The age of onset of HD symptoms is reported to occur between 15 and 21 weeks for this 61Gpb line. On the BALB/cByJ genetic background, the CAG tract remains somatically stable throughout the life span of the mouse but may contract over generations (even with male transmission). These HDexon1 mice may be useful in Huntington's Disease research.

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. Mice with this mutation were originally published on a mixed CBA x C57BL6 genetic background. It should be noted that the phenotype could vary from that originally described. We will modify the strain description as published results become available.

Development
This 1.9 kb transgene was isolated from a phage genomic clone derived from an Huntington's Disease (HD) patient and contained the 5' end of the human huntingtin (Huntington disease) gene. It was composed of approximately 1 kb of 5' UTR sequences, exon 1 (carrying expanded CAG repeats of varying size but of the order of 130 units) and the first 262 bp of intron 1. This construct was microinjected into single cell CBAxC57BL/6 embryos. Male founder R6 was bred to CBA x C57BL/6 females, producing several founder lines. Mice from founder line R6/1 have the transgene integrated as a single intact copy and were originally found to have 116 CAG repeats. At some point, these mice were backcrossed to BALB/cBy mice for at least 25 generations by Dr. Anne Messer at the Wadsworth Center (Univ. of Albany). As of 2007, the donating investigator reports that these mice carry approximately 100 CAG repeats.

Control Information

  Control
   Noncarrier
   001026 BALB/cByJ
 
  Considerations for Choosing Controls

Related Strains

View Huntington's Disease Models     (29 strains)

Strains carrying   Tg(HDexon1)61Gpb allele
006471   B6.Cg-Tg(HDexon1)61Gpb/J
002809   B6CBA-Tg(HDexon1)61Gpb/1J
View Strains carrying   Tg(HDexon1)61Gpb     (2 strains)

View Strains carrying other alleles of HTT     (12 strains)

Additional Web Information

Visit our Huntington's Disease page for a full listing of Huntington's strains and research services.
Visit the Alzheimer's Disease Mouse Model Resource site for helpful information on Alzheimer's Disease and research resources.

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.
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.

Tg(HDexon1)61Gpb/0

        involves: C57BL/6 * CBA/J
  • vision/eye phenotype
  • abnormal eye electrophysiology
    • ERG recordings at 32 weeks of age show an overall dysfunction of the retina, with a reduction of both a- and b-waves at maximal intensities   (MGI Ref ID J:80810)
    • abnormal cone electrophysiology
      • light-adapted ERG responses are completely suppressed   (MGI Ref ID J:80810)
    • abnormal rod electrophysiology
      • the dark-adapted ERG a-wave amplitude at the maximum intensity is reduced by 50% and b-wave by 70%   (MGI Ref ID J:80810)
      • a decrease in the b-wave amplitude compared with that of the a-wave indicated that rod bipolar cells postsynaptic to photoreceptors are functionally altered   (MGI Ref ID J:80810)
  • abnormal retina morphology
    • retinal dysplasia covers more than 50% of the entire retina   (MGI Ref ID J:80810)
    • progressive, late onset of retinopathy, with first histological abnormalities seen at around 16 weeks of age   (MGI Ref ID J:80810)
    • abnormal retinal outer nuclear layer morphology
      • outer nuclear layer of the retina at 32 weeks of age exhibits an irregular and wavy shape, disrupted with folds and whorls   (MGI Ref ID J:80810)
    • abnormal retinal outer plexiform layer morphology
      • misplaced photoreceptor nuclei are seen in the outer plexiform layer at 32 weeks of age   (MGI Ref ID J:80810)
      • however, no inner plexiform layer or ganglion cell layer abnormalities   (MGI Ref ID J:80810)
    • abnormal retinal photoreceptor morphology
      • misplaced photoreceptor nuclei are seen in the segment layers at 32 weeks of age   (MGI Ref ID J:80810)
      • abnormal photoreceptor inner segment morphology
        • inner segment is reduced in thickness at 32 weeks of age   (MGI Ref ID J:80810)
        • disorganized photoreceptor inner segment   (MGI Ref ID J:80810)
      • abnormal photoreceptor outer segment morphology
        • outer segment is reduced in thickness at 32 weeks of age   (MGI Ref ID J:80810)
        • disorganized photoreceptor outer segment
          • at 32 weeks of age   (MGI Ref ID J:80810)
      • retinal photoreceptor degeneration   (MGI Ref ID J:80810)
    • retinal spots
      • white spots over retina   (MGI Ref ID J:80810)
  • nervous system phenotype
  • abnormal retinal photoreceptor morphology
    • misplaced photoreceptor nuclei are seen in the segment layers at 32 weeks of age   (MGI Ref ID J:80810)
    • abnormal photoreceptor inner segment morphology
      • inner segment is reduced in thickness at 32 weeks of age   (MGI Ref ID J:80810)
      • disorganized photoreceptor inner segment   (MGI Ref ID J:80810)
    • abnormal photoreceptor outer segment morphology
      • outer segment is reduced in thickness at 32 weeks of age   (MGI Ref ID J:80810)
      • disorganized photoreceptor outer segment
        • at 32 weeks of age   (MGI Ref ID J:80810)
    • retinal photoreceptor degeneration   (MGI Ref ID J:80810)
  • neuronal intranuclear inclusions
    • nuclear inclusions are seen in the retina   (MGI Ref ID J:80810)

Tg(HDexon1)61Gpb/0

        involves: C57BL/6 * C57BL/6J * CBA
  • other phenotype
  • other aberrant phenotype
    • mice exhibit somatic and germline repeat tract expansion unlike in Neil1tm1Bjor/Neil1tm1Bjor Tg(HDexon1)61Gpb mice   (MGI Ref ID J:188338)

Tg(HDexon1)61Gpb/?

        involves: C57BL/6 * CBA
  • nervous system phenotype
  • abnormal neuron morphology
    • striatal neurons have prominent and frequent indentations of the nuclear membrane and an apparent increase in the clustering and number of nuclear pores   (MGI Ref ID J:42085)
    • increased neuron number
      • males with a CAG repeat length of 134-138 exhibit a 2-fold increase in c-Fos positive neurons in the forebrain and hindbrain   (MGI Ref ID J:203027)
    • neuronal intranuclear inclusions
      • inclusions are observed within neurons of cerebral cortex, striatum, cerebellum, spinal cord and to a much lesser degree in the hippocampus, thalamus, globus pallidus and substantia nigra   (MGI Ref ID J:42085)
      • htt immunoreactive inclusions are seen in approximately 20% of neurons   (MGI Ref ID J:42085)
      • in the striatum, ultrastructural analysis of inclusions reveals a prominent, roughly circular, pale structure   (MGI Ref ID J:42085)
      • inclusions are granular with occasional filamentous structures around the periphery; they are larger than the nucleolus and occupy 1% of nuclear volume   (MGI Ref ID J:42085)
  • abnormal striatum morphology   (MGI Ref ID J:42085)
  • behavior/neurological phenotype
  • abnormal long term spatial reference memory
    • in the last of a series of Barnes circular maze trials, the time required to locate the escape tunnel is increased in 12 week old transgenic mice as compared to wildtype   (MGI Ref ID J:130936)
    • 12 week old transgenic mice raised in an enriched environment perform as well as non-enriched wildtype in circular maze trials   (MGI Ref ID J:130936)
    • transgenic mice use both serial and spatial search strategies in the ultimate trials of the circular maze as compared to wildtype and enriched transgenic mice, which primarily use spatial search strategies   (MGI Ref ID J:130936)
  • abnormal motor capabilities/coordination/movement
    • males with 134-138 CAG repeats exhibit progressive development of motor abnormalities   (MGI Ref ID J:203027)
    • impaired coordination
      • between 8-12 weeks of age, transgenic mice perform on the rotarod as well as wildtype, however, by 14 weeks performance decreases and by 20 weeks performance is significantly impaired   (MGI Ref ID J:130936)
    • limb grasping
      • in males with 134-138 CAG repeats   (MGI Ref ID J:203027)
  • abnormal short term spatial reference memory
    • beginning at 12 weeks of age, transgenic mice exhibit a deficit in short term memory as evaluated by performance in the Y-maze   (MGI Ref ID J:130936)
  • abnormal spatial learning
    • in the last of a series of Barnes circular maze trials, the time required to locate the escape tunnel is increased in 12 week old transgenic mice as compred to wildtype   (MGI Ref ID J:130936)
    • 12 week old transgenic mice raised in an enriched environment perform as well as non-enriched wildtype in circular maze trials   (MGI Ref ID J:130936)
  • decreased exploration in new environment
    • impaired performance in location recognition test is observed by 14 weeks of age   (MGI Ref ID J:130936)
  • cardiovascular system phenotype
  • abnormal heart rate
    • males with 134-138 CAG repeats show an absence of 24 hour variation of heart rate that is seen in wild-type mice and higher heart rate levels in both the dark and light phases at 3.5 months of age, but lower heart rate during the active (dark) phase at 6.5 months of age   (MGI Ref ID J:203027)
    • 3.5 month old males with 134-138 CAG repeats exhibit a slower restoration of heart rate towards baseline in response to shake stress test   (MGI Ref ID J:203027)
    • 6.5 month old males with 134-138 CAG repeats show a tendency for a reduced peak change in heart rate response to the shake stress test and slower recovery towards the baseline   (MGI Ref ID J:203027)
    • heart rate response to the beta-adrenergic agonist isoproterenol is attenuated in 6.5 month old conscious males with 134-138 CAG repeats   (MGI Ref ID J:203027)
    • the blunted heart rate response to isoproterenol in mutants is normalized to wild-type levels by atropine pre-treatment   (MGI Ref ID J:203027)
    • abnormal sinus arrhythmia
      • males with 134-138 CAG repeats show sinus arrhythmia at 3.5 and 6.5 months of age   (MGI Ref ID J:203027)
    • ventricular tachycardia
      • in males with 134-138 CAG repeats at 3.5 and 6.5 months of age   (MGI Ref ID J:203027)
  • abnormal impulse conducting system conduction
    • males with 134-138 CAG repeats show a variety of arrhythmias at 3.5 and 6.5 months of age, including atrial-ventricular conduction blockade   (MGI Ref ID J:203027)
    • abnormal RR interval
      • males with 134-138 CAG repeats exhibit unstable RR intervals that are reversed following atropine treatment   (MGI Ref ID J:203027)
  • decreased systemic arterial blood pressure
    • males with 134-138 CAG repeats exhibit cessation of body weight gain prior to the age when they exhibit typical motor abnormalities   (MGI Ref ID J:203027)
  • irregular heartbeat
    • males with 134-138 CAG repeats exhibit unstable heart rate compared to wild-type mice, showing chaotic heart rate rhythm and irregular beat-to-beat R-R intervals   (MGI Ref ID J:203027)
    • males with 134-138 CAG repeats show a variety of arrhythmias at 3.5 and 6.5 months of age, including atrial-ventricular conduction blockade, sinus arrhythmia, atrial flutter, atrial fibrillation, supra-ventricular and ventricular premature beats, and short episodes of ventricular tachycardia   (MGI Ref ID J:203027)
    • atrial fibrillation
      • in males with 134-138 CAG repeats at 3.5 and 6.5 months of age   (MGI Ref ID J:203027)
    • ventricular premature beat
      • in males with 134-138 CAG repeats at 3.5 and 6.5 months of ag   (MGI Ref ID J:203027)
  • small myocardial fiber
    • males with 134-138 CAG repeats exhibit a smaller left ventricular cardiomyocyte diameter at 3 and 7 months of age   (MGI Ref ID J:203027)
    • however, no signs of cardiomyocyte disarray or overt fibrosis   (MGI Ref ID J:203027)
  • growth/size/body phenotype
  • decreased body weight
    • males with 134-138 CAG repeats exhibit cessation of body weight gain prior to the age when they exhibit typical motor abnormalities   (MGI Ref ID J:203027)
  • homeostasis/metabolism phenotype
  • decreased noradrenaline level
    • noradrenaline content in the left ventricle of males with 134-138 CAG repeats is 40% lower at 7 months of age   (MGI Ref ID J:203027)
  • increased circulating noradrenaline level
    • males with 134-138 CAG repeats show 6-fold higher plasma levels of noradrenaline at 7, but not 3.5, months of age   (MGI Ref ID J:203027)
  • mortality/aging
  • premature death
    • 3 of 16 males die at 6-7 months of age due to sudden cardiac death   (MGI Ref ID J:203027)
  • muscle phenotype
  • small myocardial fiber
    • males with 134-138 CAG repeats exhibit a smaller left ventricular cardiomyocyte diameter at 3 and 7 months of age   (MGI Ref ID J:203027)
    • however, no signs of cardiomyocyte disarray or overt fibrosis   (MGI Ref ID J:203027)
View Research Applications

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

Neurobiology Research
Huntington's disease

Research Tools
Neurobiology Research

HTT related

Developmental Biology Research
Neurodevelopmental Defects

Neurobiology Research
Ataxia (Movement) Defects
Behavioral and Learning Defects
Cortical Defects
Huntington's disease
Neurodegeneration
Neurodevelopmental Defects
Neurotransmitter Receptor and Synaptic Vesicle Defects
Tremor Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(HDexon1)61Gpb
Allele Name transgene insertion 61, Gillian Bates
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) HD R6/1; R6/1; httm;
Mutation Made By Gillian Bates,   United Medical and Dental Schools
Strain of OriginCBA x C57BL/6
Expressed Gene HTT, huntingtin, human
Promoter HTT, huntingtin, human
General Note The transgene is ubiquitously expressed.

Transgenic mice on a background that involves C57BL/6 and CBA display a progressive neurological phenotype that mimics many of the features of Huntington Disease in humans, including choreiform-like movements,involuntary stereotypic movements, tremor, and epileptic seizures, as well as nonmovement disorder components, including unusual vocalization.Frequent urination and loss of body weight and muscle bulk occurs through the course of the disease. Neurological developments include Neuronal Intranuclear Inclusions (NII), which contain both the huntingtin and ubiquitin proteins (NII have subsequently been identified in human HD patients). Onset of HD symptoms occurs between 15 and 21 weeks of age.

Molecular Note A human HD fragment containing a polyglutamine-repeat expansion was isolated from a clone derived from a patient with Huntington's disease. The transgene contained approximately 1 kb of 5' UTR region, exon 1 which initially contained 113 CAG repeats, and262 bp of intron 1 followed by a neomycin cassette. Subsequent analysis showed that the number of CAG repeats was prone to increase due to instability in the germline, and a range of 109.5 to 121 was observed. [MGI Ref ID J:36689]
 

Genotyping

Genotyping Information

Genotyping Protocols

TG(HDexon1), TG(YAC), Separated PCR
TG(HDexon1), TG(YAC), Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Mangiarini L; Sathasivam K; Seller M; Cozens B; Harper A; Hetherington C; Lawton M; Trottier Y; Lehrach H; Davies SW; Bates GP. 1996. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87(3):493-506. [PubMed: 8898202]  [MGI Ref ID J:36689]

Additional References

Tg(HDexon1)61Gpb related

Anglada-Huguet M; Giralt A; Perez-Navarro E; Alberch J; Xifro X. 2012. Activation of Elk-1 participates as a neuroprotective compensatory mechanism in models of Huntington's disease. J Neurochem 121(4):639-48. [PubMed: 22372926]  [MGI Ref ID J:184365]

Baiamonte BA; Lee FA; Brewer ST; Spano D; LaHoste GJ. 2013. Attenuation of Rhes activity significantly delays the appearance of behavioral symptoms in a mouse model of Huntington's disease. PLoS One 8(1):e53606. [PubMed: 23349722]  [MGI Ref ID J:195692]

Batcha AH; Greferath U; Jobling AI; Vessey KA; Ward MM; Nithianantharajah J; Hannan AJ; Kalloniatis M; Fletcher EL. 2012. Retinal dysfunction, photoreceptor protein dysregulation and neuronal remodelling in the R6/1 mouse model of Huntington's disease. Neurobiol Dis 45(3):887-96. [PubMed: 22198376]  [MGI Ref ID J:182042]

Bolivar VJ; Manley K; Messer A. 2004. Early exploratory behavior abnormalities in R6/1 Huntington's disease transgenic mice. Brain Res 1005(1-2):29-35. [PubMed: 15044061]  [MGI Ref ID J:89279]

Canals JM; Pineda JR; Torres-Peraza JF; Bosch M; Martin-Ibanez R; Munoz MT; Mengod G; Ernfors P; Alberch J. 2004. Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease. J Neurosci 24(35):7727-39. [PubMed: 15342740]  [MGI Ref ID J:92634]

Cayzac S; Delcasso S; Paz V; Jeantet Y; Cho YH. 2011. Changes in striatal procedural memory coding correlate with learning deficits in a mouse model of Huntington disease. Proc Natl Acad Sci U S A 108(22):9280-5. [PubMed: 21576479]  [MGI Ref ID J:173364]

Cha JH; Frey AS; Alsdorf SA; Kerner JA; Kosinski CM; Mangiarini L; Penney JB Jr; Davies SW; Bates GP; Young AB. 1999. Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease. Philos Trans R Soc Lond B Biol Sci 354(1386):981-9. [PubMed: 10434296]  [MGI Ref ID J:56820]

Chiang C; Jacobsen JC; Ernst C; Hanscom C; Heilbut A; Blumenthal I; Mills RE; Kirby A; Lindgren AM; Rudiger SR; McLaughlan CJ; Bawden CS; Reid SJ; Faull RL; Snell RG; Hall IM; Shen Y; Ohsumi TK; Borowsky ML; Daly MJ; Lee C; Morton CC; MacDonald ME; Gusella JF; Talkowski ME. 2012. Complex reorganization and predominant non-homologous repair following chromosomal breakage in karyotypically balanced germline rearrangements and transgenic integration. Nat Genet 44(4):390-7, S1. [PubMed: 22388000]  [MGI Ref ID J:183760]

Choi ML; Begeti F; Oh JH; Lee SY; O'Keeffe GC; Clelland CD; Tyers P; Cho ZH; Kim YB; Barker RA. 2014. Dopaminergic manipulations and its effects on neurogenesis and motor function in a transgenic mouse model of Huntington's disease. Neurobiol Dis 66:19-27. [PubMed: 24561069]  [MGI Ref ID J:212026]

Cops EJ; Sashindranath M; Daglas M; Short KM; da Fonseca Pereira C; Pang TY; Lijnen RH; Smyth IM; Hannan AJ; Samson AL; Medcalf RL. 2013. Tissue-type plasminogen activator is an extracellular mediator of Purkinje cell damage and altered gait. Exp Neurol 249:8-19. [PubMed: 23939410]  [MGI Ref ID J:203861]

Corrochano S; Renna M; Carter S; Chrobot N; Kent R; Stewart M; Cooper J; Brown SD; Rubinsztein DC; Acevedo-Arozena A. 2012. alpha-Synuclein levels modulate Huntington's disease in mice. Hum Mol Genet 21(3):485-94. [PubMed: 22010050]  [MGI Ref ID J:179605]

Crittenden JR; Dunn DE; Merali FI; Woodman B; Yim M; Borkowska AE; Frosch MP; Bates GP; Housman DE; Lo DC; Graybiel AM. 2010. CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease. Hum Mol Genet 19(9):1756-65. [PubMed: 20147317]  [MGI Ref ID J:158714]

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]

Crook ZR; Housman DE. 2012. Dysregulation of dopamine receptor D2 as a sensitive measure for Huntington disease pathology in model mice. Proc Natl Acad Sci U S A 109(19):7487-92. [PubMed: 22529362]  [MGI Ref ID J:184816]

Cummings DM; Milnerwood AJ; Dallerac GM; Waights V; Brown JY; Vatsavayai SC; Hirst MC; Murphy KP. 2006. Aberrant cortical synaptic plasticity and dopaminergic dysfunction in a mouse model of Huntington's disease. Hum Mol Genet 15(19):2856-68. [PubMed: 16905556]  [MGI Ref ID J:114924]

Cybulska-Klosowicz A; Mazarakis NK; Van Dellen A; Blakemore C; Hannan AJ; Kossut M. 2004. Impaired learning-dependent cortical plasticity in Huntington's disease transgenic mice. Neurobiol Dis 17(3):427-34. [PubMed: 15571978]  [MGI Ref ID J:94576]

Davies SW; Turmaine M; Cozens BA; DiFiglia M; Sharp AH; Ross CA ; Scherzinger E ; Wanker EE ; Mangiarini L ; Bates GP. 1997. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90(3):537-48. [PubMed: 9267033]  [MGI Ref ID J:42085]

Denny CA; Desplats PA; Thomas EA; Seyfried TN. 2010. Cerebellar lipid differences between R6/1 transgenic mice and humans with Huntington's disease. J Neurochem 115(3):748-58. [PubMed: 20731757]  [MGI Ref ID J:165859]

Desplats PA; Denny CA; Kass KE; Gilmartin T; Head SR; Sutcliffe JG; Seyfried TN; Thomas EA. 2007. Glycolipid and ganglioside metabolism imbalances in Huntington's disease. Neurobiol Dis 27(3):265-77. [PubMed: 17600724]  [MGI Ref ID J:134905]

Desplats PA; Kass KE; Gilmartin T; Stanwood GD; Woodward EL; Head SR; Sutcliffe JG; Thomas EA. 2006. Selective deficits in the expression of striatal-enriched mRNAs in Huntington's disease. J Neurochem 96(3):743-57. [PubMed: 16405510]  [MGI Ref ID J:108002]

Desplats PA; Lambert JR; Thomas EA. 2008. Functional roles for the striatal-enriched transcription factor, Bcl11b, in the control of striatal gene expression and transcriptional dysregulation in Huntington's disease. Neurobiol Dis 31(3):298-308. [PubMed: 18595722]  [MGI Ref ID J:138707]

Diaz-Hernandez M; Diez-Zaera M; Sanchez-Nogueiro J; Gomez-Villafuertes R; Canals JM; Alberch J; Miras-Portugal MT; Lucas JJ. 2009. Altered P2X7-receptor level and function in mouse models of Huntington's disease and therapeutic efficacy of antagonist administration. FASEB J 23(6):1893-906. [PubMed: 19171786]  [MGI Ref ID J:150552]

Dowie MJ; Bradshaw HB; Howard ML; Nicholson LF; Faull RL; Hannan AJ; Glass M. 2009. Altered CB1 receptor and endocannabinoid levels precede motor symptom onset in a transgenic mouse model of Huntington's disease. Neuroscience 163(1):456-65. [PubMed: 19524019]  [MGI Ref ID J:154532]

Dowie MJ; Howard ML; Nicholson LF; Faull RL; Hannan AJ; Glass M. 2010. Behavioural and molecular consequences of chronic cannabinoid treatment in Huntington's disease transgenic mice. Neuroscience 170(1):324-36. [PubMed: 20600638]  [MGI Ref ID J:165214]

Gharami K; Xie Y; An JJ; Tonegawa S; Xu B. 2008. Brain-derived neurotrophic factor over-expression in the forebrain ameliorates Huntington's disease phenotypes in mice. J Neurochem 105(2):369-79. [PubMed: 18086127]  [MGI Ref ID J:135260]

Gines S; Bosch M; Marco S; Gavalda N; Diaz-Hernandez M; Lucas JJ; Canals JM; Alberch J. 2006. Reduced expression of the TrkB receptor in Huntington's disease mouse models and in human brain. Eur J Neurosci 23(3):649-58. [PubMed: 16487146]  [MGI Ref ID J:107146]

Giralt A; Rodrigo T; Martin ED; Gonzalez JR; Mila M; Cena V; Dierssen M; Canals JM; Alberch J. 2009. Brain-derived neurotrophic factor modulates the severity of cognitive alterations induced by mutant huntingtin: involvement of phospholipaseCgamma activity and glutamate receptor expression. Neuroscience 158(4):1234-50. [PubMed: 19121372]  [MGI Ref ID J:148973]

Giralt A; Saavedra A; Carreton O; Xifro X; Alberch J; Perez-Navarro E. 2011. Increased PKA signaling disrupts recognition memory and spatial memory: role in Huntington's disease. Hum Mol Genet 20(21):4232-47. [PubMed: 21835884]  [MGI Ref ID J:176676]

Gomez GT; Hu H; McCaw EA; Denovan-Wright EM. 2006. Brain-specific factors in combination with mutant huntingtin induce gene-specific transcriptional dysregulation. Mol Cell Neurosci 31(4):661-75. [PubMed: 16446101]  [MGI Ref ID J:108616]

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]

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]

Goula AV; Stys A; Chan JP; Trottier Y; Festenstein R; Merienne K. 2012. Transcription elongation and tissue-specific somatic CAG instability. PLoS Genet 8(11):e1003051. [PubMed: 23209427]  [MGI Ref ID J:194673]

Hansson O; Castilho RF; Korhonen L; Lindholm D; Bates GP; Brundin P. 2001. Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length. J Neurochem 78(4):694-703. [PubMed: 11520890]  [MGI Ref ID J:71210]

Hansson O; Guatteo E; Mercuri NB; Bernardi G; Li XJ; Castilho RF; Brundin P. 2001. Resistance to NMDA toxicity correlates with appearance of nuclear inclusions, behavioural deficits and changes in calcium homeostasis in mice transgenic for exon 1 of the huntington gene. Eur J Neurosci 14(9):1492-504. [PubMed: 11722611]  [MGI Ref ID J:128170]

Harrison DJ; Busse M; Openshaw R; Rosser AE; Dunnett SB; Brooks SP. 2013. Exercise attenuates neuropathology and has greater benefit on cognitive than motor deficits in the R6/1 Huntington's disease mouse model. Exp Neurol 248:457-69. [PubMed: 23911978]  [MGI Ref ID J:203689]

Hathorn T; Snyder-Keller A; Messer A. 2011. Nicotinamide improves motor deficits and upregulates PGC-1alpha and BDNF gene expression in a mouse model of Huntington's disease. Neurobiol Dis 41(1):43-50. [PubMed: 20736066]  [MGI Ref ID J:167257]

Helmlinger D; Abou-Sleymane G; Yvert G; Rousseau S; Weber C; Trottier Y; Mandel JL; Devys D. 2004. Disease progression despite early loss of polyglutamine protein expression in SCA7 mouse model. J Neurosci 24(8):1881-7. [PubMed: 14985428]  [MGI Ref ID J:90121]

Helmlinger D; Yvert G; Picaud S; Merienne K; Sahel J; Mandel JL; Devys D. 2002. Progressive retinal degeneration and dysfunction in R6 Huntington's disease mice. Hum Mol Genet 11(26):3351-9. [PubMed: 12471061]  [MGI Ref ID J:80810]

Hodges A; Hughes G; Brooks S; Elliston L; Holmans P; Dunnett SB; Jones L. 2008. Brain gene expression correlates with changes in behavior in the R6/1 mouse model of Huntington's disease. Genes Brain Behav 7(3):288-99. [PubMed: 17696994]  [MGI Ref ID J:147480]

Jeantet Y; Cayzac S; Cho YH. 2013. beta oscillation during slow wave sleep and rapid eye movement sleep in the electroencephalogram of a transgenic mouse model of Huntington's disease. PLoS One 8(11):e79509. [PubMed: 24244517]  [MGI Ref ID J:209699]

Josefsen K; Nielsen MD; Jorgensen KH; Bock T; Norremolle A; Sorensen SA; Naver B; Hasholt L. 2008. Impaired glucose tolerance in the R6/1 transgenic mouse model of Huntington's disease. J Neuroendocrinol 20(2):165-72. [PubMed: 18034868]  [MGI Ref ID J:147673]

Kiriazis H; Jennings NL; Davern P; Lambert G; Su Y; Pang T; Du X; La Greca L; Head GA; Hannan AJ; Du XJ. 2012. Neurocardiac dysregulation and neurogenic arrhythmias in a transgenic mouse model of Huntington's disease. J Physiol 590(Pt 22):5845-60. [PubMed: 22890713]  [MGI Ref ID J:203027]

Kovtun IV; Liu Y; Bjoras M; Klungland A; Wilson SH; McMurray CT. 2007. OGG1 initiates age-dependent CAG trinucleotide expansion in somatic cells. Nature 447(7143):447-52. [PubMed: 17450122]  [MGI Ref ID J:122765]

Kovtun IV; Thornhill AR; McMurray CT. 2004. Somatic deletion events occur during early embryonic development and modify the extent of CAG expansion in subsequent generations. Hum Mol Genet 13(24):3057-68. [PubMed: 15496421]  [MGI Ref ID J:94584]

Kudo T; Schroeder A; Loh DH; Kuljis D; Jordan MC; Roos KP; Colwell CS. 2011. Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease. Exp Neurol 228(1):80-90. [PubMed: 21184755]  [MGI Ref ID J:170728]

Lazic SE; Goodman AO; Grote HE; Blakemore C; Morton AJ; Hannan AJ; van Dellen A; Barker RA. 2007. Olfactory abnormalities in Huntington's disease: decreased plasticity in the primary olfactory cortex of R6/1 transgenic mice and reduced olfactory discrimination in patients. Brain Res 1151:219-26. [PubMed: 17400200]  [MGI Ref ID J:122504]

Lazic SE; Grote H; Armstrong RJ; Blakemore C; Hannan AJ; van Dellen A; Barker RA. 2004. Decreased hippocampal cell proliferation in R6/1 Huntington's mice. Neuroreport 15(5):811-3. [PubMed: 15073520]  [MGI Ref ID J:89967]

Lazic SE; Grote HE; Blakemore C; Hannan AJ; van Dellen A; Phillips W; Barker RA. 2006. Neurogenesis in the R6/1 transgenic mouse model of Huntington's disease: effects of environmental enrichment. Eur J Neurosci 23(7):1829-38. [PubMed: 16623840]  [MGI Ref ID J:108068]

Li H; Wyman T; Yu ZX; Li SH; Li XJ. 2003. Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release. Hum Mol Genet 12(16):2021-30. [PubMed: 12913073]  [MGI Ref ID J:85070]

Lim NK; Hung LW; Pang TY; Mclean CA; Liddell JR; Hilton JB; Li QX; White AR; Hannan AJ; Crouch PJ. 2014. Localized changes to glycogen synthase kinase-3 and collapsin response mediator protein-2 in the Huntington's disease affected brain. Hum Mol Genet 23(15):4051-63. [PubMed: 24634145]  [MGI Ref ID J:210980]

Mangiarini L; Sathasivam K; Mahal A; Mott R; Seller M; Bates GP. 1997. Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. Nat Genet 15(2):197-200. [PubMed: 9020849]  [MGI Ref ID J:79042]

Manley K; Pugh J; Messer A. 1999. Instability of the CAG repeat in immortalized fibroblast cell cultures from Huntington's disease transgenic mice. Brain Res 835(1):74-9. [PubMed: 10448198]  [MGI Ref ID J:56438]

Manley K; Shirley TL; Flaherty L; Messer A. 1999. Msh2 deficiency prevents in vivo somatic instability of the CAG repeat in Huntington disease transgenic mice. Nat Genet 23(4):471-3. [PubMed: 10581038]  [MGI Ref ID J:58797]

Marco S; Giralt A; Petrovic MM; Pouladi MA; Martinez-Turrillas R; Martinez-Hernandez J; Kaltenbach LS; Torres-Peraza J; Graham RK; Watanabe M; Lujan R; Nakanishi N; Lipton SA; Lo DC; Hayden MR; Alberch J; Wesseling JF; Perez-Otano I. 2013. Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models. Nat Med 19(8):1030-8. [PubMed: 23852340]  [MGI Ref ID J:200090]

Mastroberardino PG; Iannicola C; Nardacci R; Bernassola F; De Laurenzi V; Melino G; Moreno S; Pavone F; Oliverio S; Fesus L; Piacentini M. 2002. 'Tissue' transglutaminase ablation reduces neuronal death and prolongs survival in a mouse model of Huntington's disease. Cell Death Differ 9(9):873-80. [PubMed: 12181738]  [MGI Ref ID J:115545]

Mazarakis NK; Cybulska-Klosowicz A; Grote H; Pang T; Van Dellen A; Kossut M; Blakemore C; Hannan AJ. 2005. Deficits in experience-dependent cortical plasticity and sensory-discrimination learning in presymptomatic Huntington's disease mice. J Neurosci 25(12):3059-66. [PubMed: 15788762]  [MGI Ref ID J:98640]

McCaw EA; Hu H; Gomez GT; Hebb AL; Kelly ME; Denovan-Wright EM. 2004. Structure, expression and regulation of the cannabinoid receptor gene (CB1) in Huntington's disease transgenic mice. Eur J Biochem 271(23-24):4909-20. [PubMed: 15606779]  [MGI Ref ID J:98030]

Miller TW; Zhou C; Gines S; MacDonald ME; Mazarakis ND; Bates GP; Huston JS; Messer A. 2005. A human single-chain Fv intrabody preferentially targets amino-terminal Huntingtin's fragments in striatal models of Huntington's disease. Neurobiol Dis 19(1-2):47-56. [PubMed: 15837560]  [MGI Ref ID J:116299]

Milnerwood AJ; Cummings DM; Dallerac GM; Brown JY; Vatsavayai SC; Hirst MC; Rezaie P; Murphy KP. 2006. Early development of aberrant synaptic plasticity in a mouse model of Huntington's disease. Hum Mol Genet 15(10):1690-703. [PubMed: 16600988]  [MGI Ref ID J:109535]

Mishra A; Dikshit P; Purkayastha S; Sharma J; Nukina N; Jana NR. 2008. E6-AP promotes misfolded polyglutamine proteins for proteasomal degradation and suppresses polyglutamine protein aggregation and toxicity. J Biol Chem 283(12):7648-56. [PubMed: 18201976]  [MGI Ref ID J:133935]

Mo C; Renoir T; Pang TY; Hannan AJ. 2013. Short-term memory acquisition in female Huntington's disease mice is vulnerable to acute stress. Behav Brain Res 253:318-22. [PubMed: 23916759]  [MGI Ref ID J:202356]

Mollersen L; Rowe AD; Illuzzi JL; Hildrestrand GA; Gerhold KJ; Tveteras L; Bjolgerud A; Wilson DM 3rd; Bjoras M; Klungland A. 2012. Neil1 is a genetic modifier of somatic and germline CAG trinucleotide repeat instability in R6/1 mice. Hum Mol Genet 21(22):4939-47. [PubMed: 22914735]  [MGI Ref ID J:188338]

Mollersen L; Rowe AD; Larsen E; Rognes T; Klungland A. 2010. Continuous and periodic expansion of CAG repeats in Huntington's disease R6/1 mice. PLoS Genet 6(12):e1001242. [PubMed: 21170307]  [MGI Ref ID J:167513]

Naver B; Stub C; Moller M; Fenger K; Hansen AK; Hasholt L; Sorensen SA. 2003. Molecular and behavioral analysis of the R6/1 Huntington's disease transgenic mouse. Neuroscience 122(4):1049-57. [PubMed: 14643771]  [MGI Ref ID J:128267]

Nicniocaill B; Haraldsson B; Hansson O; O'Connor WT; Brundin P. 2001. Altered striatal amino acid neurotransmitter release monitored using microdialysis in R6/1 Huntington transgenic mice. Eur J Neurosci 13(1):206-10. [PubMed: 11135020]  [MGI Ref ID J:128177]

Nithianantharajah J; Barkus C; Murphy M; Hannan AJ. 2008. Gene-environment interactions modulating cognitive function and molecular correlates of synaptic plasticity in Huntington's disease transgenic mice. Neurobiol Dis 29(3):490-504. [PubMed: 18165017]  [MGI Ref ID J:130936]

Nithianantharajah J; Hannan AJ. 2013. Dysregulation of synaptic proteins, dendritic spine abnormalities and pathological plasticity of synapses as experience-dependent mediators of cognitive and psychiatric symptoms in Huntington's disease. Neuroscience 251:66-74. [PubMed: 22633949]  [MGI Ref ID J:207069]

Ortiz AN; Kurth BJ; Osterhaus GL; Johnson MA. 2011. Impaired dopamine release and uptake in R6/1 Huntington's disease model mice. Neurosci Lett 492(1):11-4. [PubMed: 21256185]  [MGI Ref ID J:170713]

Pang TY; Du X; Zajac MS; Howard ML; Hannan AJ. 2009. Altered serotonin receptor expression is associated with depression-related behavior in the R6/1 transgenic mouse model of Huntington's disease. Hum Mol Genet 18(4):753-66. [PubMed: 19008301]  [MGI Ref ID J:144756]

Pang TY; Stam NC; Nithianantharajah J; Howard ML; Hannan AJ. 2006. Differential effects of voluntary physical exercise on behavioral and brain-derived neurotrophic factor expression deficits in huntington's disease transgenic mice. Neuroscience 141(2):569-84. [PubMed: 16716524]  [MGI Ref ID J:111765]

Perucho J; Casarejos MJ; Gomez A; Ruiz C; Fernandez-Estevez MA; Munoz MP; de Yebenes JG; Mena MA. 2013. Striatal infusion of glial conditioned medium diminishes huntingtin pathology in r6/1 mice. PLoS One 8(9):e73120. [PubMed: 24069174]  [MGI Ref ID J:206492]

Petersen A; Hansson O; Puschban Z; Sapp E; Romero N; Castilho RF; Sulzer D; Rice M; DiFiglia M; Przedborski S; Brundin P. 2001. Mice transgenic for exon 1 of the Huntington's disease gene display reduced striatal sensitivity to neurotoxicity induced by dopamine and 6-hydroxydopamine. Eur J Neurosci 14(9):1425-35. [PubMed: 11722604]  [MGI Ref ID J:128172]

Petersen A; Puschban Z; Lotharius J; NicNiocaill B; Wiekop P; O'Connor WT; Brundin P. 2002. Evidence for dysfunction of the nigrostriatal pathway in the R6/1 line of transgenic Huntington's disease mice. Neurobiol Dis 11(1):134-46. [PubMed: 12460553]  [MGI Ref ID J:130975]

Pietropaolo S; Delage P; Cayzac S; Crusio WE; Cho YH. 2011. Sex-dependent changes in social behaviors in motor pre-symptomatic r6/1 mice. PLoS One 6(5):e19965. [PubMed: 21603578]  [MGI Ref ID J:172743]

Pignatelli M; Lebreton F; Cho YH; Leinekugel X. 2012. "Ectopic" theta oscillations and interictal activity during slow-wave state in the R6/1 mouse model of Huntington's disease. Neurobiol Dis 48(3):409-17. [PubMed: 22842017]  [MGI Ref ID J:197506]

Pineda JR; Canals JM; Bosch M; Adell A; Mengod G; Artigas F; Ernfors P; Alberch J. 2005. Brain-derived neurotrophic factor modulates dopaminergic deficits in a transgenic mouse model of Huntington's disease. J Neurochem 93(5):1057-68. [PubMed: 15934928]  [MGI Ref ID J:99427]

Ransome MI; Hannan AJ. 2013. Impaired basal and running-induced hippocampal neurogenesis coincides with reduced Akt signaling in adult R6/1 HD mice. Mol Cell Neurosci 54:93-107. [PubMed: 23384443]  [MGI Ref ID J:203646]

Rattray I; Smith EJ; Crum WR; Walker TA; Gale R; Bates GP; Modo M. 2013. Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's disease. PLoS One 8(12):e84726. [PubMed: 24367693]  [MGI Ref ID J:211131]

Renoir T; Pang TY; Mo C; Chan G; Chevarin C; Lanfumey L; Hannan AJ. 2013. Differential effects of early environmental enrichment on emotionality related behaviours in Huntington's disease transgenic mice. J Physiol 591(Pt 1):41-55. [PubMed: 23045340]  [MGI Ref ID J:203974]

Renoir T; Zajac MS; Du X; Pang TY; Leang L; Chevarin C; Lanfumey L; Hannan AJ. 2011. Sexually dimorphic serotonergic dysfunction in a mouse model of Huntington's disease and depression. PLoS One 6(7):e22133. [PubMed: 21760962]  [MGI Ref ID J:175808]

Rozas JL; Gomez-Sanchez L; Tomas-Zapico C; Lucas JJ; Fernandez-Chacon R. 2011. Increased neurotransmitter release at the neuromuscular junction in a mouse model of polyglutamine disease. J Neurosci 31(3):1106-13. [PubMed: 21248135]  [MGI Ref ID J:168558]

Rubio I; Rodriguez-Navarro JA; Tomas-Zapico C; Ruiz C; Casarejos MJ; Perucho J; Gomez A; Rodal I; Lucas JJ; Mena MA; de Yebenes JG. 2009. Effects of partial suppression of parkin on huntingtin mutant R6/1 mice. Brain Res 1281:91-100. [PubMed: 19464273]  [MGI Ref ID J:156570]

Rue L; Lopez-Soop G; Gelpi E; Martinez-Vicente M; Alberch J; Perez-Navarro E. 2013. Brain region- and age-dependent dysregulation of p62 and NBR1 in a mouse model of Huntington's disease. Neurobiol Dis 52:219-28. [PubMed: 23295856]  [MGI Ref ID J:197643]

Saavedra A; Garcia-Martinez JM; Xifro X; Giralt A; Torres-Peraza JF; Canals JM; Diaz-Hernandez M; Lucas JJ; Alberch J; Perez-Navarro E. 2010. PH domain leucine-rich repeat protein phosphatase 1 contributes to maintain the activation of the PI3K/Akt pro-survival pathway in Huntington's disease striatum. Cell Death Differ 17(2):324-35. [PubMed: 19745829]  [MGI Ref ID J:169436]

Saavedra A; Giralt A; Arumi H; Alberch J; Perez-Navarro E. 2013. Regulation of hippocampal cGMP levels as a candidate to treat cognitive deficits in Huntington's disease. PLoS One 8(9):e73664. [PubMed: 24040016]  [MGI Ref ID J:207349]

Saavedra A; Giralt A; Rue L; Xifro X; Xu J; Ortega Z; Lucas JJ; Lombroso PJ; Alberch J; Perez-Navarro E. 2011. Striatal-Enriched Protein Tyrosine Phosphatase Expression and Activity in Huntington's Disease: A STEP in the Resistance to Excitotoxicity. J Neurosci 31(22):8150-8162. [PubMed: 21632937]  [MGI Ref ID J:173377]

Smith R; Chung H; Rundquist S; Maat-Schieman ML; Colgan L; Englund E; Liu YJ; Roos RA; Faull RL; Brundin P; Li JY. 2006. Cholinergic neuronal defect without cell loss in Huntington's disease. Hum Mol Genet 15(21):3119-31. [PubMed: 16987871]  [MGI Ref ID J:114849]

Smith R; Petersen A; Bates GP; Brundin P; Li JY. 2005. Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction. Neurobiol Dis 20(3):673-84. [PubMed: 15967669]  [MGI Ref ID J:104634]

Spires TL; Grote HE; Garry S; Cordery PM; Van Dellen A; Blakemore C; Hannan AJ. 2004. Dendritic spine pathology and deficits in experience-dependent dendritic plasticity in R6/1 Huntington's disease transgenic mice. Eur J Neurosci 19(10):2799-807. [PubMed: 15147313]  [MGI Ref ID J:90295]

Spires TL; Grote HE; Varshney NK; Cordery PM; van Dellen A; Blakemore C; Hannan AJ. 2004. Environmental enrichment rescues protein deficits in a mouse model of Huntington's disease, indicating a possible disease mechanism. J Neurosci 24(9):2270-6. [PubMed: 14999077]  [MGI Ref ID J:90128]

Teles AV; Rosenstock TR; Okuno CS; Lopes GS; Bertoncini CR; Smaili SS. 2008. Increase in bax expression and apoptosis are associated in Huntington's disease progression. Neurosci Lett 438(1):59-63. [PubMed: 18468793]  [MGI Ref ID J:136970]

Tomas-Zapico C; Diez-Zaera M; Ferrer I; Gomez-Ramos P; Moran MA; Miras-Portugal MT; Diaz-Hernandez M; Lucas JJ. 2012. alpha-Synuclein accumulates in huntingtin inclusions but forms independent filaments and its deficiency attenuates early phenotype in a mouse model of Huntington's disease. Hum Mol Genet 21(3):495-510. [PubMed: 22045698]  [MGI Ref ID J:179713]

Tome S; Manley K; Simard JP; Clark GW; Slean MM; Swami M; Shelbourne PF; Tillier ER; Monckton DG; Messer A; Pearson CE. 2013. MSH3 polymorphisms and protein levels affect CAG repeat instability in Huntington's disease mice. PLoS Genet 9(2):e1003280. [PubMed: 23468640]  [MGI Ref ID J:195193]

Vatsavayai SC; Dallerac GM; Milnerwood AJ; Cummings DM; Rezaie P; Murphy KP; Hirst MC. 2007. Progressive CAG expansion in the brain of a novel R6/1-89Q mouse model of Huntington's disease with delayed phenotypic onset. Brain Res Bull 72(2-3):98-102. [PubMed: 17352932]  [MGI Ref ID J:128652]

Xifro X; Giralt A; Saavedra A; Garcia-Martinez JM; Diaz-Hernandez M; Lucas JJ; Alberch J; Perez-Navarro E. 2009. Reduced calcineurin protein levels and activity in exon-1 mouse models of Huntington's disease: role in excitotoxicity. Neurobiol Dis 36(3):461-9. [PubMed: 19733666]  [MGI Ref ID J:155500]

Yano H; Baranov SV; Baranova OV; Kim J; Pan Y; Yablonska S; Carlisle DL; Ferrante RJ; Kim AH; Friedlander RM. 2014. Inhibition of mitochondrial protein import by mutant huntingtin. Nat Neurosci 17(6):822-31. [PubMed: 24836077]  [MGI Ref ID J:212904]

Yu ZX; Li SH; Evans J; Pillarisetti A; Li H; Li XJ. 2003. Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. J Neurosci 23(6):2193-202. [PubMed: 12657678]  [MGI Ref ID J:82676]

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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.

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Breeding & HusbandryWhen maintaining a live colony, hemizygous mice are bred to BALB/cByJ inbred mice or to wildtype siblings.

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Cryorecovery* $2525.00
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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.

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    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).

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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.

Standard Supply

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

Supply Notes

  • 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).

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Standard Supply

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

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"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.

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