Strain Name:

B6CBA-Tg(HDexon1)62Gpb/3J

Stock Number:

006494

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Level 3

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These transgenic mice display a progressive neurological phenotype that mimics many of the features of Huntington Disease (HD) 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, which contain both the huntingtin and ubiquitin proteins. Onset of HD symptoms occurs between 15 and 21 weeks of age. This line is transgenic for the 5' end of the human HD gene carrying approximately 120 +/- 5 (CAG)repeat expansions.

Description

Strain Information

Type Mutant Stock; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
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Breeding Considerations This strain is a good breeder.
Specieslaboratory mouse
GenerationN58 (14-AUG-14)
Generation Definitions
 
Donating Investigator Gillian P Bates,   United Medical and Dental Schools

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Appearance
black
Related Genotype: a/a

agouti, ataxic, tremors
Related Genotype: A/? HTT/-

agouti
Related Genotype: A/?

black, ataxic,tremors
Related Genotype: a/a HTT/-

Important Note
January 2007: alteration in strain name and phenotype. Please see Strain Development for additional information.

Description
This line is transgenic for the 5' end of the human HD gene carrying approximately 120 +/- 5 (CAG)repeat expansions. 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. Previously unknown, these NII have subsequently been identified in human HD patients. The age of onset of HD symptoms is reported to occur between 9 and 11 weeks. Commonly known as the "R6/2" strain.

Transgenic mice develop hyperglycemia by 12 weeks of age with a corresponding decrease in insulin levels. Pancreatic beta cells develop huntingtin inclusions as early as 7 weeks of age, by 12 weeks more than 95% of beta cells have inclusions. Pancreatic alpha and delta cells also exhibit some inclusions (24% and 6% of cells, respectively) by 12 weeks. Pancreatic islets become hypotonic and beta cells are dramatically reduced in number by 12 weeks. Beta cells contain very few insulin secretory vesicles. (Bjorkquvist M., et al. 2005)

Sequence analysis identified the transgene insertion site within an intron of the predicted gene Gm12695 on chromosome 4. Relative to the forward strand, the exact insertion site is 3' of chr4:96,409,480 bp (assembly NCBI 37/mus musuculus 9) (Cowin R-M et al., 2012 PLoS ONE 6(12):e28409).

This strain ships with a JAXTagTM affixed. Learn more about JAXTagTM.

Development
The transgene is about 1 kb of the human HD gene and includes the promoter region, exon 1 with 120 +/- 5 CAG repeats. This strain was identified as the R6-2 line in the original publication.

In fall 2006, the B6CBA-Tg(HDexon1)62Gpb/1J colony was shown to have a reduction in the number of CAG repeat units in the transgene (approximately 100-105 CAG repeats), and to exhibit a concomitant decrease in severity and delayed onset in the expected neurological phenotype. The Repository recovered frozen embryos from the HDexon1 line during the winter of 2006/7. The cryo-recovered line has approximately 160 +/- 10 CAG repeats, and onset of HD symptoms occurs between 9 and 11 weeks of age, as originally reported for this strain.

The cryo-recovered line continues as B6CBA-Tg(HDexon1)62Gpb/1J (Stock No. 002810). The phenotypically changed line formerly distributed as Stock No. 002810 is now named B6CBA-Tg(HDexon1)62Gpb/3J (Stock No. 006494) and is maintained with a CAG repeat of 120 +/- 5 repeat units.

Sequence analysis identified the transgene insertion site within an intron of the predicted gene Gm12695 on chromosome 4. Relative to the forward strand, the exact insertion site is 3' of chr4:96,409,480 bp (assembly NCBI 37/mus musuculus 9) (Cowin R-M et al., 2012 PLoS ONE 6(12):e28409).

Control Information

  Control
   Noncarrier
 
  Considerations for Choosing Controls

Related Strains

View Huntington's Disease Models     (29 strains)

Strains carrying   Tg(HDexon1)62Gpb allele
002810   B6CBA-Tg(HDexon1)62Gpb/1J
View Strains carrying   Tg(HDexon1)62Gpb     (1 strain)

View Strains carrying other alleles of HTT     (13 strains)

Additional Web Information

A Field Guide to Working with Mouse Models of Huntington's Disease. Working jointly with CHDI and PsychoGenics, Inc., we now have this valuable resource available for you.
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
- 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

Tg(HDexon1)62Gpb/0

        involves: C57BL/6J * CBA/J
  • mortality/aging
  • premature death
    • usually between 10 to 13 weeks of age   (MGI Ref ID J:36689)
    • mice can die suddenly   (MGI Ref ID J:36689)
  • nervous system phenotype
  • decreased brain size
    • average of 19% smaller than wildtype   (MGI Ref ID J:36689)
  • seizures
    • handling induced seizures that can be several minutes duration   (MGI Ref ID J:36689)
  • behavior/neurological phenotype
  • abnormal vocalization
    • 2 distinct characteristic vocalizations observed   (MGI Ref ID J:36689)
  • impaired balance
    • mice lose balance when sitting on hind limbs, turning and grooming their backs   (MGI Ref ID J:36689)
  • limb grasping
    • dyskinesia of limbs when suspended by the tail   (MGI Ref ID J:36689)
  • seizures
    • handling induced seizures that can be several minutes duration   (MGI Ref ID J:36689)
  • stereotypic behavior
    • onset of motor impairment as early as 4 weeks of age   (MGI Ref ID J:36689)
    • stereotypic repetitive stroking of the nose and face, hind limb kicking and scratching movement   (MGI Ref ID J:36689)
  • tremors
    • progressive resting tremor in limbs, trunk and head   (MGI Ref ID J:36689)
    • involuntary jerky shudders   (MGI Ref ID J:36689)
  • endocrine/exocrine gland phenotype
  • abnormal pancreatic alpha cell morphology
    • islets exhibit huntingtin inclusions in 24% of cells by 12 weeks of age   (MGI Ref ID J:96353)
  • abnormal pancreatic beta cell morphology
    • islets exhibit huntingtin inclusions in 19% of cells by 7 weeks of age, by week 12 frequency is greater than 95%   (MGI Ref ID J:96353)
    • islets are hypotrophic by 12 weeks, BrdU incorporation is reduced 6-fold   (MGI Ref ID J:96353)
    • significant reduction in insulin and somatostatin content by 12 weeks   (MGI Ref ID J:96353)
    • islets are smaller in size by 12 weeks   (MGI Ref ID J:96353)
    • islets contain few insulin secretory vesicles by 12 weeks   (MGI Ref ID J:96353)
    • decreased pancreatic beta cell number
      • by 12 weeks, no signs of apoptosis or necrosis are observed   (MGI Ref ID J:96353)
  • abnormal pancreatic delta cell morphology
    • islets exhibit huntingtin inclusions in 6% of cells by 12 weeks of age   (MGI Ref ID J:96353)
  • small ovary   (MGI Ref ID J:36689)
  • small seminal vesicle
    • small seminal ducts and gland size   (MGI Ref ID J:36689)
  • small testis   (MGI Ref ID J:36689)
  • growth/size/body phenotype
  • decreased body weight
    • body weight declines after 10 weeks   (MGI Ref ID J:96353)
    • weight loss
      • body weight is normal at weaning   (MGI Ref ID J:36689)
      • with progression of phenotype up to 30% of body weight can be lost   (MGI Ref ID J:36689)
      • overall loss of muscle bulk observed   (MGI Ref ID J:36689)
  • homeostasis/metabolism phenotype
  • abnormal glucose homeostasis
    • abnormal glucose homeostasis   (MGI Ref ID J:96353)
    • decreased circulating insulin level
      • insulin secretion in response to glucose and KCl is decreased 4-fold and 2.5-fold, respectively, by 12 weeks of age   (MGI Ref ID J:96353)
    • impaired glucose tolerance
      • identified at 11.5 weeks of age, insulin response absent   (MGI Ref ID J:96353)
  • renal/urinary system phenotype
  • polyuria   (MGI Ref ID J:36689)
  • reproductive system phenotype
  • abnormal female reproductive system morphology
    • smaller size ovaries and uteri often observed   (MGI Ref ID J:36689)
    • small ovary   (MGI Ref ID J:36689)
    • small uterus   (MGI Ref ID J:36689)
  • female infertility   (MGI Ref ID J:36689)
  • small seminal vesicle
    • small seminal ducts and gland size   (MGI Ref ID J:36689)
  • small testis   (MGI Ref ID J:36689)

Tg(HDexon1)62Gpb/0

        involves: C57BL/6 * CBA
  • nervous system phenotype
  • abnormal cerebral cortex morphology
    • immunoreactive htt is detected in the cortex beginning at 3.5 weeks   (MGI Ref ID J:42085)
  • 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 by 10-12 weeks of age   (MGI Ref ID J:42085)
    • 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)
      • inclusions appear in the cerebral cortex before they can be detected in the striatum   (MGI Ref ID J:42085)
      • inclusions are ubiquitinated by 5-6 weeks and can be detected by ultrastructural analysis by 8 weeks   (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
    • immunoreactive htt is detected in the striatum at 4.5 weeks   (MGI Ref ID J:42085)
  • decreased brain weight
    • reduction in brain weight by 5 weeks   (MGI Ref ID J:42085)
  • growth/size/body phenotype
  • decreased body weight
    • progressive loss of body weight   (MGI Ref ID J:42085)

Tg(HDexon1)62Gpb/0

        B6CBA-Tg(HDexon1)62Gpb/3J
  • behavior/neurological phenotype
  • abnormal gait
    • wider base (separation between legs) than controls; females exhibit difference earlier than males   (MGI Ref ID J:185262)
    • short stride length
      • decreased stride length (ipsilateral) observed at 8 weeks of age   (MGI Ref ID J:185262)
      • shorter splay length (contralateral) than controls   (MGI Ref ID J:185262)
  • decreased grip strength
    • progressive deterioration of grip strength especially in males between 10-14 weeks of age   (MGI Ref ID J:185262)
  • decreased startle reflex
    • beginning at 4 weeks of age   (MGI Ref ID J:185262)
  • decreased vertical activity
    • mice rear less in the center of the open field test than controls at all ages in the light phase and by 6 weeks of age in the dark phase   (MGI Ref ID J:185262)
    • mice exhibit a progressive decrease in climbing activity starting at 4 weeks of age   (MGI Ref ID J:185262)
  • hypoactivity
    • mice are hypoactive in the open field test in both the light and dark phases of the light cycle   (MGI Ref ID J:185262)
    • in the light phase females are less active at 8 and 14 weeks of age   (MGI Ref ID J:185262)
    • in the light phase males are less active starting at 6 weeks of age   (MGI Ref ID J:185262)
    • in the dark phase both sexes are hypoactive beginning at 12 weeks of age   (MGI Ref ID J:185262)
    • progressive hypoactivity is observed in the center of the open field beginning at 4 weeks of age   (MGI Ref ID J:185262)
  • impaired coordination
    • mice exhibit a reduced latency to fall on rotarod test beginning at 4 weeks of age as compared to wild-type   (MGI Ref ID J:185262)
    • latency time decreases with age   (MGI Ref ID J:185262)
  • increased anxiety-related response
    • increased preference for dark in dark/light choice test at 12 and 14 weeks of age   (MGI Ref ID J:185262)
  • growth/size/body phenotype
  • decreased body weight
    • decrease in body weight is observed by 7 weeks in both genders   (MGI Ref ID J:185262)
  • mortality/aging
  • premature death
    • median survival of 113 days   (MGI Ref ID J:185262)
    • all mice die within 12 weeks of the first death   (MGI Ref ID J:185262)
  • nervous system phenotype
  • decreased prepulse inhibition
    • observed after 8 weeks of age   (MGI Ref ID J:185262)

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

Tg(HDexon1)62Gpb/0

        involves: C57BL/6 * CBA/J
  • vision/eye phenotype
  • abnormal retina morphology
    • retinal dysplasia covers more than 50% of the entire retina   (MGI Ref ID J:80810)
    • abnormal retinal outer nuclear layer morphology
      • outer nuclear layer of the retina exhibits an irregular and wavy shape, disrupted with folds and whorls at 10 weeks of age   (MGI Ref ID J:80810)
    • abnormal retinal outer plexiform layer morphology
      • misplaced photoreceptor nuclei are seen in the outer plexiform layer at 10 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 10 weeks of age   (MGI Ref ID J:80810)
      • abnormal photoreceptor inner segment morphology
        • inner segment is reduced in thickness at 10 weeks of age   (MGI Ref ID J:80810)
        • disorganized photoreceptor inner segment
          • seen at 10 weeks of age   (MGI Ref ID J:80810)
      • abnormal photoreceptor outer segment morphology
        • outer segment is reduced in thickness at 10 weeks of age   (MGI Ref ID J:80810)
        • disorganized photoreceptor outer segment
          • seen at 10 weeks of age   (MGI Ref ID J:80810)
      • retinal photoreceptor degeneration   (MGI Ref ID J:80810)
  • nervous system phenotype
  • abnormal retinal photoreceptor morphology
    • misplaced photoreceptor nuclei are seen in the segment layers at 10 weeks of age   (MGI Ref ID J:80810)
    • abnormal photoreceptor inner segment morphology
      • inner segment is reduced in thickness at 10 weeks of age   (MGI Ref ID J:80810)
      • disorganized photoreceptor inner segment
        • seen at 10 weeks of age   (MGI Ref ID J:80810)
    • abnormal photoreceptor outer segment morphology
      • outer segment is reduced in thickness at 10 weeks of age   (MGI Ref ID J:80810)
      • disorganized photoreceptor outer segment
        • seen at 10 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)62Gpb/0

        B6.Cg-Tg(HDexon1)62Gpb/240
  • behavior/neurological phenotype
  • abnormal gait
    • narrower base (separation between legs) than controls   (MGI Ref ID J:185262)
    • short stride length
      • shorter splay length (contralateral) than controls at 14 weeks of age   (MGI Ref ID J:185262)
      • stride length (ipsilateral) is similar to controls   (MGI Ref ID J:185262)
  • decreased grip strength
    • progressive deterioration of grip strength especially in males at 14 weeks of age   (MGI Ref ID J:185262)
  • decreased startle reflex
    • observed at 6 and 14 weeks of age in both genders   (MGI Ref ID J:185262)
  • decreased vertical activity
    • mice rear less in the center of the open field test than controls starting at 12 weeks of age in the light phase   (MGI Ref ID J:185262)
    • rearing is similar to controls in dark phase   (MGI Ref ID J:185262)
  • hypoactivity
    • mice cover less total distance in light phase of open field test starting at 6 weeks of age in females and 8 weeks of age in males   (MGI Ref ID J:185262)
    • in dark phase, hypoactivity is significant at 6 weeks of age in both genders   (MGI Ref ID J:185262)
    • mice cover less distance in center starting at 12 weeks in light phase and 6 weeks in dark phase   (MGI Ref ID J:185262)
  • impaired coordination
    • mice exhibit a progressive impairment on the rotarod test beginning at 8 weeks of age   (MGI Ref ID J:185262)
  • increased anxiety-related response
    • increased preference for dark in dark/light choice test at 14 weeks of age   (MGI Ref ID J:185262)
  • growth/size/body phenotype
  • decreased body weight
    • body weight decreases by 10 weeks of age in females and 9 weeks in males   (MGI Ref ID J:185262)
  • mortality/aging
  • premature death
    • median survival of 141 days for females and 179 days for males   (MGI Ref ID J:185262)
    • all mice die within 9 weeks of the first death   (MGI Ref ID J:185262)
  • nervous system phenotype
  • decreased prepulse inhibition
    • observed at 14 weeks of age, especially in females   (MGI Ref ID J:185262)

Tg(HDexon1)62Gpb/0

        involves: C57BL/6 * C57BL/6JOlaHsd * CBA * CBA/CaOlaHsd
  • behavior/neurological phenotype
  • decreased grip strength   (MGI Ref ID J:193012)
  • impaired coordination   (MGI Ref ID J:193012)
  • nervous system phenotype
  • decreased brain weight   (MGI Ref ID J:193012)
View Research Applications

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

Cardiovascular Research

Diabetes and Obesity Research
Hyperglycemia
      adult onset
Hypoinsulinemia
      adult onset
Type 2 Diabetes (NIDDM)
      adult onset

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)62Gpb
Allele Name transgene insertion 62, Gillian Bates
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) R6/2; R6/2B; Tg(HDexon1)62nGpb;
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 Transgenic mice exhibit a progressive neurological phenotype that mimics many of the features of HD. Onset of phenotype is apparent from approximately 8 weeks of age based on home cage behavior. Some functional tests indicate the presence of a motor impairment from 5-6 weeks and cognitive impairment from 3 weeks. Epileptic seizures are seen in a small percentage of transgenic mice. A failure to gain weight is more pronounced in males than females. Immunohistochemistry with antibodies raised against the N-terminus of huntingtin reveals aggregates in the form of intranuclear inclusions and neuropil aggregates.

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, 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); the onset of HD symptoms occurs between 9 and 11 weeks.

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 142 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 when inherited through the male line due to instability in the germline. A range of 141 to 157 was observed. On a background that involves C57BL/6 and CBA, transgneic mice have been observed to carry >(CAG)200 repeat expansions. The insertion site has been localized to a position on mouse chromosome 4 in an intron of predicted gene GM12695. The transgene is ubiquitously expressed. [MGI Ref ID J:181024] [MGI Ref ID J:36689]
 

Genotyping

Genotyping Information

Genotyping Protocols

Generic Pde6b, High Resolution Melting
Generic Pde6b, Standard PCR
HDexon Hi T CAG, Fluorescent PCR
HDexon1 62Gpb repeat, Fluorescent 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]

Menalled L; El-Khodor BF; Patry M; Suarez-Farinas M; Orenstein SJ; Zahasky B; Leahy C; Wheeler V; Yang XW; MacDonald M; Morton AJ; Bates G; Leeds J; Park L; Howland D; Signer E; Tobin A; Brunner D. 2009. Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models. Neurobiol Dis 35(3):319-36. [PubMed: 19464370]  [MGI Ref ID J:185262]

Additional References

Tg(HDexon1)62Gpb related

Acevedo-Torres K; Berrios L; Rosario N; Dufault V; Skatchkov S; Eaton MJ; Torres-Ramos CA; Ayala-Torres S. 2009. Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's disease. DNA Repair (Amst) 8(1):126-36. [PubMed: 18935984]  [MGI Ref ID J:144556]

Andre VM; Cepeda C; Venegas A; Gomez Y; Levine MS. 2006. Altered cortical glutamate receptor function in the R6/2 model of Huntington's disease. J Neurophysiol 95(4):2108-19. [PubMed: 16381805]  [MGI Ref ID J:128683]

Andreassen OA; Dedeoglu A; Stanojevic V; Hughes DB; Browne SE; Leech CA; Ferrante RJ; Habener JF; Beal MF; Thomas MK. 2002. Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expression. Neurobiol Dis 11(3):410-24. [PubMed: 12586550]  [MGI Ref ID J:127926]

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]

Ariano MA; Cepeda C; Calvert CR; Flores-Hernandez J; Hernandez-Echeagaray E; Klapstein GJ; Chandler SH; Aronin N; DiFiglia M; Levine MS. 2005. Striatal potassium channel dysfunction in Huntington's disease transgenic mice. J Neurophysiol 93(5):2565-74. [PubMed: 15625098]  [MGI Ref ID J:128569]

Bailey CD; Johnson GV. 2005. Tissue transglutaminase contributes to disease progression in the R6/2 Huntington's disease mouse model via aggregate-independent mechanisms. J Neurochem 92(1):83-92. [PubMed: 15606898]  [MGI Ref ID J:95452]

Banez-Coronel M; Porta S; Kagerbauer B; Mateu-Huertas E; Pantano L; Ferrer I; Guzman M; Estivill X; Marti E. 2012. A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity. PLoS Genet 8(2):e1002481. [PubMed: 22383888]  [MGI Ref ID J:183404]

Batista CM; Kippin TE; Willaime-Morawek S; Shimabukuro MK; Akamatsu W; van der Kooy D. 2006. A progressive and cell non-autonomous increase in striatal neural stem cells in the Huntington's disease R6/2 mouse. J Neurosci 26(41):10452-60. [PubMed: 17035529]  [MGI Ref ID J:113257]

Beck H; Flynn K; Lindenberg KS; Schwarz H; Bradke F; Di Giovanni S; Knoll B. 2012. Serum Response Factor (SRF)-cofilin-actin signaling axis modulates mitochondrial dynamics. Proc Natl Acad Sci U S A 109(38):E2523-32. [PubMed: 22927399]  [MGI Ref ID J:190033]

Benn CL; Butler R; Mariner L; Nixon J; Moffitt H; Mielcarek M; Woodman B; Bates GP. 2009. Genetic knock-down of HDAC7 does not ameliorate disease pathogenesis in the R6/2 mouse model of Huntington's disease. PLoS One 4(6):e5747. [PubMed: 19484127]  [MGI Ref ID J:150207]

Benn CL; Sun T; Sadri-Vakili G; McFarland KN; DiRocco DP; Yohrling GJ; Clark TW; Bouzou B; Cha JH. 2008. Huntingtin modulates transcription, occupies gene promoters in vivo, and binds directly to DNA in a polyglutamine-dependent manner. J Neurosci 28(42):10720-33. [PubMed: 18923047]  [MGI Ref ID J:141086]

Benraiss A; Toner MJ; Xu Q; Bruel-Jungerman E; Rogers EH; Wang F; Economides AN; Davidson BL; Kageyama R; Nedergaard M; Goldman SA. 2013. Sustained mobilization of endogenous neural progenitors delays disease progression in a transgenic model of Huntington's disease. Cell Stem Cell 12(6):787-99. [PubMed: 23746982]  [MGI Ref ID J:199086]

Bett JS; Cook C; Petrucelli L; Bates GP. 2009. The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease. PLoS ONE 4(4):e5128. [PubMed: 19352500]  [MGI Ref ID J:148097]

Bibb JA; Yan Z; Svenningsson P; Snyder GL; Pieribone VA; Horiuchi A; Nairn AC; Messer A; Greengard P. 2000. Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice. Proc Natl Acad Sci U S A 97(12):6809-14. [PubMed: 10829080]  [MGI Ref ID J:62714]

Bjorkqvist M; Fex M; Renstrom E; Wierup N; Petersen A; Gil J; Bacos K; Popovic N; Li JY; Sundler F; Brundin P; Mulder H. 2005. The R6/2 transgenic mouse model of Huntington's disease develops diabetes due to deficient beta-cell mass and exocytosis. Hum Mol Genet 14(5):565-74. [PubMed: 15649949]  [MGI Ref ID J:96353]

Bjorkqvist M; Petersen A; Bacos K; Isaacs J; Norlen P; Gil J; Popovic N; Sundler F; Bates GP; Tabrizi SJ; Brundin P; Mulder H. 2006. Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease. Hum Mol Genet 15(10):1713-21. [PubMed: 16613897]  [MGI Ref ID J:109534]

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]

Bobrowska A; Donmez G; Weiss A; Guarente L; Bates G. 2012. SIRT2 ablation has no effect on tubulin acetylation in brain, cholesterol biosynthesis or the progression of Huntington's disease phenotypes in vivo. PLoS One 7(4):e34805. [PubMed: 22511966]  [MGI Ref ID J:193012]

Bobrowska A; Paganetti P; Matthias P; Bates GP. 2011. Hdac6 Knock-Out Increases Tubulin Acetylation but Does Not Modify Disease Progression in the R6/2 Mouse Model of Huntington's Disease. PLoS One 6(6):e20696. [PubMed: 21677773]  [MGI Ref ID J:174136]

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Rossi S; Prosperetti C; Picconi B; De Chiara V; Mataluni G; Bernardi G; Calabresi P; Centonze D. 2006. Deficits of glutamate transmission in the striatum of toxic and genetic models of Huntington's disease. Neurosci Lett 410(1):6-10. [PubMed: 17070651]  [MGI Ref ID J:144664]

Rudenko O; Tkach V; Berezin V; Bock E. 2009. Detection of early behavioral markers of Huntington's disease in R6/2 mice employing an automated social home cage. Behav Brain Res 203(2):188-99. [PubMed: 19410605]  [MGI Ref ID J:150372]

Rudenko O; Tkach V; Berezin V; Bock E. 2010. Effects of FGF receptor peptide agonists on animal behavior under normal and pathological conditions. Neurosci Res 68(1):35-43. [PubMed: 20562017]  [MGI Ref ID J:164072]

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]

Sadagurski M; Cheng Z; Rozzo A; Palazzolo I; Kelley GR; Dong X; Krainc D; White MF. 2011. IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease. J Clin Invest 121(10):4070-81. [PubMed: 21926467]  [MGI Ref ID J:178490]

Samadi P; Boutet A; Rymar VV; Rawal K; Maheux J; Kvann JC; Tomaszewski M; Beaubien F; Cloutier JF; Levesque D; Sadikot AF. 2013. Relationship between BDNF expression in major striatal afferents, striatum morphology and motor behavior in the R6/2 mouse model of Huntington's disease. Genes Brain Behav 12(1):108-24. [PubMed: 23006318]  [MGI Ref ID J:206780]

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]

Sathyasaikumar KV; Stachowski EK; Amori L; Guidetti P; Muchowski PJ; Schwarcz R. 2010. Dysfunctional kynurenine pathway metabolism in the R6/2 mouse model of Huntington's disease. J Neurochem 113(6):1416-25. [PubMed: 20236387]  [MGI Ref ID J:163576]

Sawiak SJ; Wood NI; Carpenter TA; Morton AJ. 2012. Huntington's disease mouse models online: high-resolution MRI images with stereotaxic templates for computational neuroanatomy. PLoS One 7(12):e53361. [PubMed: 23300918]  [MGI Ref ID J:195721]

Sawiak SJ; Wood NI; Williams GB; Morton AJ; Carpenter TA. 2009. Use of magnetic resonance imaging for anatomical phenotyping of the R6/2 mouse model of Huntington's disease. Neurobiol Dis 33(1):12-9. [PubMed: 18930823]  [MGI Ref ID J:144563]

Sawiak SJ; Wood NI; Williams GB; Morton AJ; Carpenter TA. 2009. Voxel-based morphometry in the R6/2 transgenic mouse reveals differences between genotypes not seen with manual 2D morphometry. Neurobiol Dis 33(1):20-7. [PubMed: 18930824]  [MGI Ref ID J:144562]

Sbodio JI; Paul BD; Machamer CE; Snyder SH. 2013. Golgi protein ACBD3 mediates neurotoxicity associated with Huntington's disease. Cell Rep 4(5):890-7. [PubMed: 24012756]  [MGI Ref ID J:202841]

Schipper-Krom S; Juenemann K; Jansen AH; Wiemhoefer A; van den Nieuwendijk R; Smith DL; Hink MA; Bates GP; Overkleeft H; Ovaa H; Reits E. 2014. Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies. FEBS Lett 588(1):151-9. [PubMed: 24291262]  [MGI Ref ID J:208072]

Seo H; Kim W; Isacson O. 2008. Compensatory changes in the ubiquitin-proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice partially model Huntington's disease patients. Hum Mol Genet 17(20):3144-53. [PubMed: 18640989]  [MGI Ref ID J:139795]

She P; Zhang Z; Marchionini D; Diaz WC; Jetton TJ; Kimball SR; Vary TC; Lang CH; Lynch CJ. 2011. Molecular characterization of skeletal muscle atrophy in the R6/2 mouse model of Huntington's disease. Am J Physiol Endocrinol Metab 301(1):E49-61. [PubMed: 21505144]  [MGI Ref ID J:182068]

Simmons DA; Casale M; Alcon B; Pham N; Narayan N; Lynch G. 2007. Ferritin accumulation in dystrophic microglia is an early event in the development of Huntington's disease. Glia 55(10):1074-84. [PubMed: 17551926]  [MGI Ref ID J:156308]

Smith MR; Syed A; Lukacsovich T; Purcell J; Barbaro BA; Worthge SA; Wei SR; Pollio G; Magnoni L; Scali C; Massai L; Franceschini D; Camarri M; Gianfriddo M; Diodato E; Thomas R; Gokce O; Tabrizi SJ; Caricasole A; Landwehrmeyer B; Menalled L; Murphy C; Ramboz S; Luthi-Carter R; Westerberg G; Marsh JL. 2014. A potent and selective Sirtuin 1 inhibitor alleviates pathology in multiple animal and cell models of Huntington's disease. Hum Mol Genet 23(11):2995-3007. [PubMed: 24436303]  [MGI Ref ID J:210364]

Smith R; Bacos K; Fedele V; Soulet D; Walz HA; Obermuller S; Lindqvist A; Bjorkqvist M; Klein P; Onnerfjord P; Brundin P; Mulder H; Li JY. 2009. Mutant huntingtin interacts with {beta}-tubulin and disrupts vesicular transport and insulin secretion. Hum Mol Genet 18(20):3942-54. [PubMed: 19628478]  [MGI Ref ID J:152918]

Snider BJ; Moss JL; Revilla FJ; Lee CS; Wheeler VC; Macdonald ME; Choi DW. 2003. Neocortical neurons cultured from mice with expanded CAG repeats in the huntingtin gene: unaltered vulnerability to excitotoxins and other insults. Neuroscience 120(3):617-25. [PubMed: 12895502]  [MGI Ref ID J:128151]

Stack EC; Dedeoglu A; Smith KM; Cormier K; Kubilus JK; Bogdanov M; Matson WR; Yang L; Jenkins BG; Luthi-Carter R; Kowall NW; Hersch SM; Beal MF; Ferrante RJ. 2007. Neuroprotective effects of synaptic modulation in Huntington's disease R6/2 mice. J Neurosci 27(47):12908-15. [PubMed: 18032664]  [MGI Ref ID J:127643]

Stack EC; Kubilus JK; Smith K; Cormier K; Del Signore SJ; Guelin E; Ryu H; Hersch SM; Ferrante RJ. 2005. Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice. J Comp Neurol 490(4):354-70. [PubMed: 16127709]  [MGI Ref ID J:102810]

Steele AD; Jackson WS; King OD; Lindquist S. 2007. The power of automated high-resolution behavior analysis revealed by its application to mouse models of Huntington's and prion diseases Proc Natl Acad Sci U S A 104(6):1983-1988. [PubMed: 17261803]  [MGI Ref ID J:117096]

Strand AD; Baquet ZC; Aragaki AK; Holmans P; Yang L; Cleren C; Beal MF; Jones L; Kooperberg C; Olson JM; Jones KR. 2007. Expression profiling of Huntington's disease models suggests that brain-derived neurotrophic factor depletion plays a major role in striatal degeneration. J Neurosci 27(43):11758-68. [PubMed: 17959817]  [MGI Ref ID J:127037]

Sun Z; Del Mar N; Meade C; Goldowitz D; Reiner A. 2002. Differential changes in striatal projection neurons in R6/2 transgenic mice for Huntington's disease. Neurobiol Dis 11(3):369-85. [PubMed: 12586547]  [MGI Ref ID J:127927]

Sun Z; Wang HB; Deng YP; Lei WL; Xie JP; Meade CA; Del Mar N; Goldowitz D; Reiner A. 2005. Increased calbindin-D28k immunoreactivity in striatal projection neurons of R6/2 Huntington's disease transgenic mice. Neurobiol Dis 20(3):907-17. [PubMed: 15990326]  [MGI Ref ID J:104657]

Tabrizi SJ; Workman J; Hart PE; Mangiarini L; Mahal A; Bates G; Cooper JM; Schapira AH. 2000. Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse Ann Neurol 47(1):80-6. [PubMed: 10632104]  [MGI Ref ID J:60060]

Tallaksen-Greene SJ; Janiszewska A; Benton K; Ruprecht L; Albin RL. 2010. Lack of efficacy of NMDA receptor-NR2B selective antagonists in the R6/2 model of Huntington disease. Exp Neurol 225(2):402-7. [PubMed: 20659453]  [MGI Ref ID J:165304]

Tarditi A; Camurri A; Varani K; Borea PA; Woodman B; Bates G; Cattaneo E; Abbracchio MP. 2006. Early and transient alteration of adenosine A2A receptor signaling in a mouse model of Huntington disease. Neurobiol Dis 23(1):44-53. [PubMed: 16651003]  [MGI Ref ID J:111115]

Thomas EA; Coppola G; Desplats PA; Tang B; Soragni E; Burnett R; Gao F; Fitzgerald KM; Borok JF; Herman D; Geschwind DH; Gottesfeld JM. 2008. The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice. Proc Natl Acad Sci U S A 105(40):15564-9. [PubMed: 18829438]  [MGI Ref ID J:141829]

Thomas EA; Coppola G; Tang B; Kuhn A; Kim S; Geschwind DH; Brown TB; Luthi-Carter R; Ehrlich ME. 2011. In vivo cell-autonomous transcriptional abnormalities revealed in mice expressing mutant huntingtin in striatal but not cortical neurons. Hum Mol Genet 20(6):1049-60. [PubMed: 21177255]  [MGI Ref ID J:168853]

Tkac I; Dubinsky JM; Keene CD; Gruetter R; Low WC. 2007. Neurochemical changes in Huntington R6/2 mouse striatum detected by in vivo 1H NMR spectroscopy. J Neurochem 100(5):1397-406. [PubMed: 17217418]  [MGI Ref ID J:141746]

Tong X; Ao Y; Faas GC; Nwaobi SE; Xu J; Haustein MD; Anderson MA; Mody I; Olsen ML; Sofroniew MV; Khakh BS. 2014. Astrocyte Kir4.1 ion channel deficits contribute to neuronal dysfunction in Huntington's disease model mice. Nat Neurosci 17(5):694-703. [PubMed: 24686787]  [MGI Ref ID J:212770]

Traficante A; Riozzi B; Cannella M; Rampello L; Squitieri F; Battaglia G. 2007. Reduced activity of cortico-striatal fibres in the R6/2 mouse model of Huntington's disease. Neuroreport 18(18):1997-2000. [PubMed: 18007201]  [MGI Ref ID J:128502]

Tsoi H; Lau TC; Tsang SY; Lau KF; Chan HY. 2012. CAG expansion induces nucleolar stress in polyglutamine diseases. Proc Natl Acad Sci U S A 109(33):13428-33. [PubMed: 22847428]  [MGI Ref ID J:188784]

Valenza M; Leoni V; Karasinska JM; Petricca L; Fan J; Carroll J; Pouladi MA; Fossale E; Nguyen HP; Riess O; MacDonald M; Wellington C; DiDonato S; Hayden M; Cattaneo E. 2010. Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes. J Neurosci 30(32):10844-50. [PubMed: 20702713]  [MGI Ref ID J:163232]

Valenza M; Leoni V; Tarditi A; Mariotti C; Bjorkhem I; Di Donato S; Cattaneo E. 2007. Progressive dysfunction of the cholesterol biosynthesis pathway in the R6/2 mouse model of Huntington's disease. Neurobiol Dis 28(1):133-42. [PubMed: 17702587]  [MGI Ref ID J:134836]

Vetter JM; Jehle T; Heinemeyer J; Franz P; Behrens PF; Jackisch R; Landwehrmeyer GB; Feuerstein TJ. 2003. Mice transgenic for exon 1 of Huntington's disease: properties of cholinergic and dopaminergic pre-synaptic function in the striatum. J Neurochem 85(4):1054-63. [PubMed: 12716437]  [MGI Ref ID J:83389]

Wacker JL; Huang SY; Steele AD; Aron R; Lotz GP; Nguyen Q; Giorgini F; Roberson ED; Lindquist S; Masliah E; Muchowski PJ. 2009. Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease. J Neurosci 29(28):9104-14. [PubMed: 19605647]  [MGI Ref ID J:151692]

Wade A; Jacobs P; Morton AJ. 2008. Atrophy and degeneration in sciatic nerve of presymptomatic mice carrying the Huntington's disease mutation. Brain Res 1188:61-8. [PubMed: 18062944]  [MGI Ref ID J:130502]

Walker AG; Miller BR; Fritsch JN; Barton SJ; Rebec GV. 2008. Altered information processing in the prefrontal cortex of Huntington's disease mouse models. J Neurosci 28(36):8973-82. [PubMed: 18768691]  [MGI Ref ID J:141171]

Walker AG; Ummel JR; Rebec GV. 2011. Reduced expression of conditioned fear in the R6/2 mouse model of Huntington's disease is related to abnormal activity in prelimbic cortex. Neurobiol Dis 43(2):379-87. [PubMed: 21515374]  [MGI Ref ID J:173207]

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]

Wang CE; Zhou H; McGuire JR; Cerullo V; Lee B; Li SH; Li XJ. 2008. Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin. J Cell Biol 181(5):803-16. [PubMed: 18504298]  [MGI Ref ID J:137025]

Wang X; Zhu S; Pei Z; Drozda M; Stavrovskaya IG; Del Signore SJ; Cormier K; Shimony EM; Wang H; Ferrante RJ; Kristal BS; Friedlander RM. 2008. Inhibitors of cytochrome c release with therapeutic potential for Huntington's disease. J Neurosci 28(38):9473-85. [PubMed: 18799679]  [MGI Ref ID J:142757]

Waters CW; Varuzhanyan G; Talmadge RJ; Voss AA. 2013. Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction. Proc Natl Acad Sci U S A 110(22):9160-5. [PubMed: 23671115]  [MGI Ref ID J:197426]

Weydt P; Pineda VV; Torrence AE; Libby RT; Satterfield TF; Lazarowski ER; Gilbert ML; Morton GJ; Bammler TK; Strand AD; Cui L; Beyer RP; Easley CN; Smith AC; Krainc D; Luquet S; Sweet IR; Schwartz MW; La Spada AR. 2006. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. Cell Metab 4(5):349-62. [PubMed: 17055784]  [MGI Ref ID J:129751]

Williams RH; Morton AJ; Burdakov D. 2011. Paradoxical function of orexin/hypocretin circuits in a mouse model of Huntington's disease. Neurobiol Dis 42(3):438-45. [PubMed: 21324360]  [MGI Ref ID J:172764]

Wood NI; Carta V; Milde S; Skillings EA; McAllister CJ; Ang YL; Duguid A; Wijesuriya N; Afzal SM; Fernandes JX; Leong TW; Morton J. 2010. Responses to environmental enrichment differ with sex and genotype in a transgenic mouse model of Huntington's disease. PLoS One 5(2):e9077. [PubMed: 20174443]  [MGI Ref ID J:157990]

Wood NI; Glynn D; Morton AJ. 2011. 'Brain training' improves cognitive performance and survival in a transgenic mouse model of Huntington's disease. Neurobiol Dis 42(3):427-37. [PubMed: 21324361]  [MGI Ref ID J:172763]

Wood NI; McAllister CJ; Cuesta M; Aungier J; Fraenkel E; Morton AJ. 2013. Adaptation to experimental jet-lag in R6/2 mice despite circadian dysrhythmia. PLoS One 8(2):e55036. [PubMed: 23390510]  [MGI Ref ID J:199429]

Wood NI; Pallier PN; Wanderer J; Morton AJ. 2007. Systemic administration of Congo red does not improve motor or cognitive function in R6/2 mice. Neurobiol Dis 25(2):342-53. [PubMed: 17095235]  [MGI Ref ID J:119012]

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]

Xiang Z; Valenza M; Cui L; Leoni V; Jeong HK; Brilli E; Zhang J; Peng Q; Duan W; Reeves SA; Cattaneo E; Krainc D. 2011. Peroxisome-Proliferator-Activated Receptor Gamma Coactivator 1 {alpha} Contributes to Dysmyelination in Experimental Models of Huntington's Disease. J Neurosci 31(26):9544-9553. [PubMed: 21715619]  [MGI Ref ID J:174060]

Yohrling GJ 4th; Jiang GC; DeJohn MM; Miller DW; Young AB; Vrana KE; Cha JH. 2003. Analysis of cellular, transgenic and human models of Huntington's disease reveals tyrosine hydroxylase alterations and substantia nigra neuropathology. Brain Res Mol Brain Res 119(1):28-36. [PubMed: 14597227]  [MGI Ref ID J:132442]

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 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]

Zarringhalam K; Ka M; Kook YH; Terranova JI; Suh Y; King OD; Um M. 2012. An open system for automatic home-cage behavioral analysis and its application to male and female mouse models of Huntington's disease. Behav Brain Res 229(1):216-25. [PubMed: 22266926]  [MGI Ref ID J:181876]

Zhang Y; Li M; Drozda M; Chen M; Ren S; Mejia Sanchez RO; Leavitt BR; Cattaneo E; Ferrante RJ; Hayden MR; Friedlander RM. 2003. Depletion of wild-type huntingtin in mouse models of neurologic diseases. J Neurochem 87(1):101-6. [PubMed: 12969257]  [MGI Ref ID J:135605]

Zhang Y; Ona VO; Li M; Drozda M; Dubois-Dauphin M; Przedborski S; Ferrante RJ; Friedlander RM. 2003. Sequential activation of individual caspases, and of alterations in Bcl-2 proapoptotic signals in a mouse model of Huntington's disease. J Neurochem 87(5):1184-92. [PubMed: 14622098]  [MGI Ref ID J:86809]

Zourlidou A; Gidalevitz T; Kristiansen M; Landles C; Woodman B; Wells DJ; Latchman DS; de Belleroche J; Tabrizi SJ; Morimoto RI; Bates GP. 2007. Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation. Hum Mol Genet 16(9):1078-90. [PubMed: 17360721]  [MGI Ref ID J:121760]

Zwilling D; Huang SY; Sathyasaikumar KV; Notarangelo FM; Guidetti P; Wu HQ; Lee J; Truong J; Andrews-Zwilling Y; Hsieh EW; Louie JY; Wu T; Scearce-Levie K; Patrick C; Adame A; Giorgini F; Moussaoui S; Laue G; Rassoulpour A; Flik G; Huang Y; Muchowski JM;Masliah E; Schwarcz R; Muchowski PJ. 2011. Kynurenine 3-monooxygenase inhibition in blood ameliorates neurodegeneration. Cell 145(6):863-74. [PubMed: 21640374]  [MGI Ref ID J:173374]

van der Burg JM; Bacos K; Wood NI; Lindqvist A; Wierup N; Woodman B; Wamsteeker JI; Smith R; Deierborg T; Kuhar MJ; Bates GP; Mulder H; Erlanson-Albertsson C; Morton AJ; Brundin P; Petersen A; Bjorkqvist M. 2008. Increased metabolism in the R6/2 mouse model of Huntington's disease. Neurobiol Dis 29(1):41-51. [PubMed: 17920283]  [MGI Ref ID J:141483]

van der Burg JM; Winqvist A; Aziz NA; Maat-Schieman ML; Roos RA; Bates GP; Brundin P; Bjorkqvist M; Wierup N. 2011. Gastrointestinal dysfunction contributes to weight loss in Huntington's disease mice. Neurobiol Dis :. [PubMed: 21624468]  [MGI Ref ID J:173319]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           MP13

Colony Maintenance

Breeding & HusbandryHemizygous females are not fertile. Hemizygous males have a 3-4 week breeding window so mating scheme should be via multiple females. Also, only about half of the male hemizygotes are fertile. The breeding scheme was: B6CBAF1 females X hemizygous HD62 males, preferably a trio (2 B6CBAF1 females and one hemizygous male). Strain is now maintained by ovarian transplant hemizygote females x B6CBAF1/J males. Both mating schemes are available to the customer, but OT hemi female x B6CBAF1/J is recommended mating scheme. Expected coat color from breeding:Black, Agouti
Breeding Considerations This strain is a good breeder.
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Live Mice

Weeks of AgePrice per mouse (US dollars $)GenderGenotypes Provided
5 weeks $281.50Female or MaleHemizygous for Tg(HDexon1)62Gpb  
6 weeks $286.95Female or MaleHemizygous for Tg(HDexon1)62Gpb  
7 weeks $292.40Female or MaleHemizygous for Tg(HDexon1)62Gpb  
8 weeks $297.85Female or MaleHemizygous for Tg(HDexon1)62Gpb  
Price per Pair (US dollars $)Pair Genotype
$316.20Hemizygous for Tg(HDexon1)62Gpb (ovary transplant) x B6CBAF1/J (100011)  

Standard Supply

Level 3. Up to 50 mice. Larger quantities or custom orders arranged upon request.

Supply Notes

  • This strain ships with a JAXTagTM affixed. Learn more about JAXTagTM.
  • Shipped at a specific age in weeks. Mice at a precise age in days and littermates are also available.
  • Strains that must be genotyped are not available until five to seven weeks of age.
Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Weeks of AgePrice per mouse (US dollars $)GenderGenotypes Provided
5 weeks $366.00Female or MaleHemizygous for Tg(HDexon1)62Gpb  
6 weeks $373.10Female or MaleHemizygous for Tg(HDexon1)62Gpb  
7 weeks $380.20Female or MaleHemizygous for Tg(HDexon1)62Gpb  
8 weeks $387.30Female or MaleHemizygous for Tg(HDexon1)62Gpb  
Price per Pair (US dollars $)Pair Genotype
$411.10Hemizygous for Tg(HDexon1)62Gpb (ovary transplant) x B6CBAF1/J (100011)  

Standard Supply

Level 3. Up to 50 mice. Larger quantities or custom orders arranged upon request.

Supply Notes

  • This strain ships with a JAXTagTM affixed. Learn more about JAXTagTM.
  • Shipped at a specific age in weeks. Mice at a precise age in days and littermates are also available.
  • Strains that must be genotyped are not available until five to seven weeks of age.
  • Strains that require genotyping are only offered at five weeks of age and older. The time required for sample collection, assay, reporting, and completion of USDA documentation required for international purchases make distribution of younger mice prohibitive. Mice at a precise age in days, littermates and retired breeders are also available.
View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Level 3. Up to 50 mice. Larger quantities or custom orders arranged upon request.

Control Information

  Control
   Noncarrier
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Important Note

January 2007: alteration in strain name and phenotype. Please see Strain Development for additional information.

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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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"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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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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