Strain Name:

B6CBA-Tg(HDexon1)62Gpb/1J

Stock Number:

002810

Availability:

Level 4

Use Restrictions Apply, see Terms of Use

Description

Strain Information

Former Names B6CBA-Tg(HDexon1)62oGpb/J    (Changed: 15-DEC-04 )
B6CBA-TgN(HDexon1)62Gpb    (Changed: 15-DEC-04 )
Type Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
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Specieslaboratory mouse
GenerationN30 (20-DEC-06)
 
Donating Investigator Gillian Bates,   United Medical and Dental Schools

Appearance
black
Related Genotype: a/a

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

agouti
Related Genotype: A/?

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

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

Description
This line is transgenic for the 5' end of the human HD gene carrying (CAG)115-(CAG)150 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 nine 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 seven 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)

Development
The transgene is about 1 kb of the human HD gene and includes the promoter region, exon 1 with 154-59 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 100-115 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 154 to 159 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 will be named B6CBA-Tg(HDexon1)62Gpb/1J (Stock No. 002810). The line formerly distributed as Stock No. 002810 will be named B6CBA-Tg(HDexon1)62Gpb/3J (Stock No. 006494) and distributed as long as there is sufficient demand.

Control Information

  Control
   Noncarrier
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Tg(HDexon1)62Gpb allele
006494   B6CBA-Tg(HDexon1)62Gpb/3J
View Strains carrying   Tg(HDexon1)62Gpb     (1 strain)

View Strains carrying other alleles of HTT     (9 strains)

Additional Web Information

Genetic Quality Control Annual Report

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

View Mammalian Phenotype Terms

Mammalian Phenotype Terms
      assigned by genotype

Tg(HDexon1)62Gpb/0

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

Tg(HDexon1)62Gpb/0

        B6CBA-Tg(HDexon1)62Gpb/1J
  • cardiovascular system phenotype
  • abnormal cardiac stroke volume (MGI Ref ID J:116660)
    • mice exhibit progressive reduction in stroke volume, although there are no differences in heart rate
  • abnormal diastolic filling velocity (MGI Ref ID J:116660)
    • transmitral flow velocity is altered as compared to controls
    • E/A ratio is significantly decreased at all time points
    • mice exhibit a decrease in passive ventricular filling and a significant increase in atrial-mediated left ventricle filling
  • abnormal myocardial fiber morphology (MGI Ref ID J:116660)
    • mitochondria in cardiomyocytes examined at 12 weeks of age exhibit a circular appearance with a disruption of uniform densities
  • decreased cardiac output (MGI Ref ID J:116660)
    • detected as early as 8 weeks of age and reduced to 50% by 12 weeks of age
  • decreased heart weight (MGI Ref ID J:116660)
    • significantly reduced at 12 weeks of age
  • decreased left ventricle systolic pressure (MGI Ref ID J:116660)
    • diminished left ventricular wall motion during systole
    • left ventricle fractional shortening significantly impaired at 10 and 12 weeks of age
  • decreased ventricle muscle contractility (MGI Ref ID J:116660)
    • diminished left ventricular wall motion during systole
    • left ventricle fractional shortening significantly impaired at 10 and 12 weeks of age
  • dilated left ventricle (MGI Ref ID J:116660)
    • mice exhibit left ventricular dilation in comparison to controls
  • growth/size phenotype
  • postnatal slow weight gain (MGI Ref ID J:116660)
    • mice exhibited less weight gain over time than controls
  • weight loss (MGI Ref ID J:99425)
  • muscle phenotype
  • abnormal myocardial fiber morphology (MGI Ref ID J:116660)
    • mitochondria in cardiomyocytes examined at 12 weeks of age exhibit a circular appearance with a disruption of uniform densities
  • decreased ventricle muscle contractility (MGI Ref ID J:116660)
    • diminished left ventricular wall motion during systole
    • left ventricle fractional shortening significantly impaired at 10 and 12 weeks of age
  • behavior/neurological phenotype
  • abnormal voluntary movement (MGI Ref ID J:99425)
    • impaired mobility in home cages
  • limb grasping (MGI Ref ID J:99425)
    • exhibit clasping of both fore and hind paws
  • nervous system phenotype
  • abnormal cerebellum morphology (MGI Ref ID J:111237)
    • 3-HK levels are significantly elevated in the cerebellum at 4 weeks of age, a phenotype observed in Huntington disease patients
  • abnormal cerebral cortex morphology (MGI Ref ID J:111237)
    • 3-HK levels are significantly elevated in the cortex at 4 weeks of age, a phenotype observed in Huntington disease patients
  • abnormal nervous system physiology (MGI Ref ID J:99425)
    • striatal and cortical mitochondria are equally resistant to calcium at 8 and 12 weeks of age, while in controls, striatal mitochondria is more sensitive to calcium
    • abnormal neuron physiology (MGI Ref ID J:76018)
      • striatal and cortical neurons display more rapid and increased swelling to N-methyl-D-aspartate (NMDA) than controls, but not to alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) or kainate (KA), indicating increased sensitivity to NMDA
      • intracellular recordings show that resting membrane potentials of striatal neurons are significantly more depolarized than in controls
  • abnormal striatum morphology (MGI Ref ID J:111237)
    • 3-hydroxykynurenine (3-HK) levels are significantly elevated in the striatum at 4 weeks of age, a phenotype observed in Huntington disease patients

Tg(HDexon1)62Gpb/0

        involves: C57BL/6 * CBA
  • nervous system phenotype
  • abnormal cerebral cortex morphology (MGI Ref ID J:42085)
    • immunoreactive htt is detected in the cortex beginning at 3.5 weeks
  • abnormal neuron morphology (MGI Ref ID J:42085)
    • 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
    • neuronal intranuclear inclusions (MGI Ref ID J:42085)
      • 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
      • inclusions appear in the cerebral cortex before they can be detected in the striatum
      • inclusions are ubiquitinated by 5-6 weeks and can be detected by ultrastructural analysis by 8 weeks
      • htt immunoreactive inclusions are seen in approximately 20% of neurons
      • in the striatum, ultrastructural analysis of inclusions reveals a prominent, roughly circular, pale structure
      • inclusions are granular with occasional filamentous structures around the periphery; they are larger than the nucleolus and occupy 1% of nuclear volume
  • abnormal striatum morphology (MGI Ref ID J:42085)
    • immunoreactive htt is detected in the striatum at 4.5 weeks
  • decreased brain weight (MGI Ref ID J:42085)
    • reduction in brain weight by 5 weeks
  • growth/size phenotype
  • decreased body weight (MGI Ref ID J:42085)
    • progressive loss of body weight

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

Tg(HDexon1)62Gpb/0

        involves: C57BL/6 * CBA/J * SJL
  • vision/eye phenotype
  • abnormal eye morphology (MGI Ref ID J:80810)
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

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

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

 
Allele Symbol Tg(HDexon1)62Gpb
Allele Name transgene insertion 62, Gillian Bates
Allele Type Transgenic (random, expressed)
Common Name(s) R6/2; 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 obseved to carry >(CAG)200 repeat expansions. The transgene is ubiquitously expressed. [MGI Ref ID J:36689]
 

Genotyping

Genotyping Information

Genotyping Protocols

(HDexon1)62Gpb, STD PCR, vers. 1
Generic RD Melt Curve Analysis, MCA, vers. 2
Generic RD, STD PCR, vers. 1
TG(HDexon1), TG(YAC), STD PCR, vers. 1

Helpful Links

Optimizing PCR Protocols

References

References

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

Bogdanov MB; Andreassen OA; Dedeoglu A; Ferrante RJ; Beal MF. 2001. Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease. J Neurochem 79(6):1246-9. [PubMed: 11752065]  [MGI Ref ID J:73480]

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]

Dedeoglu A; Kubilus JK; Jeitner TM; Matson SA; Bogdanov M; Kowall NW; Matson WR; Cooper AJ; Ratan RR; Beal MF; Hersch SM; Ferrante RJ. 2002. Therapeutic effects of cystamine in a murine model of Huntington's disease. J Neurosci 22(20):8942-50. [PubMed: 12388601]  [MGI Ref ID J:79781]

Dedeoglu A; Kubilus JK; Yang L; Ferrante KL; Hersch SM; Beal MF; Ferrante RJ. 2003. Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice. J Neurochem 85(6):1359-67. [PubMed: 12787055]  [MGI Ref ID J:83796]

Fain JN; Del Mar NA; Meade CA; Reiner A; Goldowitz D. 2001. Abnormalities in the functioning of adipocytes from R6/2 mice that are transgenic for the Huntington's disease mutation. Hum Mol Genet 10(2):145-52. [PubMed: 11152662]  [MGI Ref ID J:67073]

Ferrante RJ; Kubilus JK; Lee J; Ryu H; Beesen A; Zucker B; Smith K; Kowall NW; Ratan RR; Luthi-Carter R; Hersch SM. 2003. Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice. J Neurosci 23(28):9418-27. [PubMed: 14561870]  [MGI Ref ID J:88202]

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]

Karpuj MV; Becher MW; Springer JE; Chabas D; Youssef S; Pedotti R; Mitchell D; Steinman L. 2002. Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine. Nat Med 8(2):143-9. [PubMed: 11821898]  [MGI Ref ID J:74541]

Li H; Li SH; Yu ZX; Shelbourne P; Li XJ. 2001. Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice. J Neurosci 21(21):8473-81. [PubMed: 11606636]  [MGI Ref ID J:72357]

Lievens JC; Woodman B; Mahal A; Spasic-Boscovic O; Samuel D; Kerkerian-Le Goff L; Bates GP. 2001. Impaired glutamate uptake in the r6 huntington's disease transgenic mice. Neurobiol Dis 8(5):807-21. [PubMed: 11592850]  [MGI Ref ID J:72696]

Luthi-Carter R; Hanson SA; Strand AD; Bergstrom DA; Chun W; Peters NL; Woods AM; Chan EY; Kooperberg C; Krainc D; Young AB; Tapscott SJ; Olson JM. 2002. Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain. Hum Mol Genet 11(17):1911-26. [PubMed: 12165554]  [MGI Ref ID J:78591]

Luthi-Carter R; Strand A; Peters NL; Solano SM; Hollingsworth ZR; Menon AS; Frey AS; Spektor BS; Penney EB; Schilling G; Ross CA; Borchelt DR; Tapscott SJ; Young AB; Cha JH; Olson JM. 2000. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Hum Mol Genet 9(9):1259-71. [PubMed: 10814708]  [MGI Ref ID J:62544]

Meade CA; Deng YP; Fusco FR; Del Mar N; Hersch S; Goldowitz D; Reiner A. 2002. Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice. J Comp Neurol 449(3):241-69. [PubMed: 12115678]  [MGI Ref ID J:77468]

Menalled L; Zanjani H; MacKenzie L; Koppel A; Carpenter E; Zeitlin S; Chesselet MF. 2000. Decrease in striatal enkephalin mRNA in mouse models of Huntington's disease. Exp Neurol 162(2):328-42. [PubMed: 10739639]  [MGI Ref ID J:61687]

Mihm MJ; Amann DM; Schanbacher BL; Altschuld RA; Bauer JA; Hoyt KR. 2007. Cardiac dysfunction in the R6/2 mousse model of Huntington's disease Neurobiol Dis 25(2):297-308. [PubMed: 17126554]  [MGI Ref ID J:116660]

Nagaoka U; Kim K; Jana NR; Doi H; Maruyama M; Mitsui K; Oyama F; Nukina N. 2004. Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions. J Neurochem 91(1):57-68. [PubMed: 15379887]  [MGI Ref ID J:93278]

Rebec GV; Barton SJ; Ennis MD. 2002. Dysregulation of ascorbate release in the striatum of behaving mice expressing the Huntington's disease gene. J Neurosci 22(2):RC202. [PubMed: 11784814]  [MGI Ref ID J:79994]

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]

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]

Tg(HDexon1)62Gpb related

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]

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]

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]

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]

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]

Bogdanov MB; Andreassen OA; Dedeoglu A; Ferrante RJ; Beal MF. 2001. Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease. J Neurochem 79(6):1246-9. [PubMed: 11752065]  [MGI Ref ID J:73480]

Brustovetsky N; LaFrance R; Purl KJ; Brustovetsky T; Keene CD; Low WC; Dubinsky JM. 2005. Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's Disease. J Neurochem 93(6):1361-70. [PubMed: 15935052]  [MGI Ref ID J:99425]

Cepeda C; Hurst RS; Calvert CR; Hernandez-Echeagaray E; Nguyen OK; Jocoy E; Christian LJ; Ariano MA; Levine MS. 2003. Transient and progressive electrophysiological alterations in the corticostriatal pathway in a mouse model of Huntington's disease. J Neurosci 23(3):961-9. [PubMed: 12574425]  [MGI Ref ID J:81916]

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

Choo YS; Mao Z; Johnson GV; Lesort M. 2005. Increased glutathione levels in cortical and striatal mitochondria of the R6/2 Huntington's disease mouse model. Neurosci Lett 386(1):63-8. [PubMed: 15993538]  [MGI Ref ID J:102620]

Ciamei A; Morton AJ. 2008. Rigidity in social and emotional memory in the R6/2 mouse model of Huntington's disease. Neurobiol Learn Mem 89(4):533-44. [PubMed: 18069020]  [MGI Ref ID J:139616]

Cipriani S; Bizzoco E; Gianfriddo M; Melani A; Vannucchi MG; Pedata F. 2008. Adenosine A2A receptor antagonism increases nNOS-immunoreactive neurons in the striatum of Huntington transgenic mice. Exp Neurol 213(1):163-70. [PubMed: 18586241]  [MGI Ref ID J:138610]

Conforti P; Ramos C; Apostol BL; Simmons DA; Nguyen HP; Riess O; Thompson LM; Zuccato C; Cattaneo E. 2008. Blood level of brain-derived neurotrophic factor mRNA is progressively reduced in rodent models of Huntington's disease: restoration by the neuroprotective compound CEP-1347. Mol Cell Neurosci 39(1):1-7. [PubMed: 18571429]  [MGI Ref ID J:141758]

Crocker SF; Costain WJ; Robertson HA. 2006. DNA microarray analysis of striatal gene expression in symptomatic transgenic Huntington's mice (R6/2) reveals neuroinflammation and insulin associations. Brain Res 1088(1):176-86. [PubMed: 16626669]  [MGI Ref ID J:109666]

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]

Deckel AW; Gordinier A; Nuttal D; Tang V; Kuwada C; Freitas R; Gary KA. 2001. Reduced activity and protein expression of NOS in R6/2 HD transgenic mice: effects of L-NAME on symptom progression. Brain Res 919(1):70-81. [PubMed: 11689164]  [MGI Ref ID J:72845]

Dedeoglu A; Kubilus JK; Jeitner TM; Matson SA; Bogdanov M; Kowall NW; Matson WR; Cooper AJ; Ratan RR; Beal MF; Hersch SM; Ferrante RJ. 2002. Therapeutic effects of cystamine in a murine model of Huntington's disease. J Neurosci 22(20):8942-50. [PubMed: 12388601]  [MGI Ref ID J:79781]

Dedeoglu A; Kubilus JK; Yang L; Ferrante KL; Hersch SM; Beal MF; Ferrante RJ. 2003. Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice. J Neurochem 85(6):1359-67. [PubMed: 12787055]  [MGI Ref ID J:83796]

Doi H; Okamura K; Bauer PO; Furukawa Y; Shimizu H; Kurosawa M; Machida Y; Miyazaki H; Mitsui K; Kuroiwa Y; Nukina N. 2008. RNA-binding protein TLS is a major nuclear aggregate-interacting protein in huntingtin exon 1 with expanded polyglutamine-expressing cells. J Biol Chem 283(10):6489-500. [PubMed: 18167354]  [MGI Ref ID J:133683]

Fain JN; Del Mar NA; Meade CA; Reiner A; Goldowitz D. 2001. Abnormalities in the functioning of adipocytes from R6/2 mice that are transgenic for the Huntington's disease mutation. Hum Mol Genet 10(2):145-52. [PubMed: 11152662]  [MGI Ref ID J:67073]

Ferrante RJ; Andreassen OA; Dedeoglu A; Ferrante KL; Jenkins BG; Hersch SM; Beal MF. 2002. Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. J Neurosci 22(5):1592-9. [PubMed: 11880489]  [MGI Ref ID J:75156]

Ferrante RJ; Kubilus JK; Lee J; Ryu H; Beesen A; Zucker B; Smith K; Kowall NW; Ratan RR; Luthi-Carter R; Hersch SM. 2003. Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice. J Neurosci 23(28):9418-27. [PubMed: 14561870]  [MGI Ref ID J:88202]

Fox JH; Barber DS; Singh B; Zucker B; Swindell MK; Norflus F; Buzescu R; Chopra R; Ferrante RJ; Kazantsev A; Hersch SM. 2004. Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation. J Neurochem 91(2):413-22. [PubMed: 15447674]  [MGI Ref ID J:93257]

Fujimoto M; Takaki E; Hayashi T; Kitaura Y; Tanaka Y; Inouye S; Nakai A. 2005. Active HSF1 significantly suppresses polyglutamine aggregate formation in cellular and mouse models. J Biol Chem 280(41):34908-16. [PubMed: 16051598]  [MGI Ref ID J:102465]

Gianfriddo M; Melani A; Turchi D; Giovannini MG; Pedata F. 2004. Adenosine and glutamate extracellular concentrations and mitogen-activated protein kinases in the striatum of Huntington transgenic mice. Selective antagonism of adenosine A2A receptors reduces transmitter outflow. Neurobiol Dis 17(1):77-88. [PubMed: 15350968]  [MGI Ref ID J:127868]

Gibson HE; Reim K; Brose N; Morton AJ; Jones S. 2005. A similar impairment in CA3 mossy fibre LTP in the R6/2 mouse model of Huntington's disease and in the complexin II knockout mouse. Eur J Neurosci 22(7):1701-12. [PubMed: 16197510]  [MGI Ref ID J:102923]

Gil JM; Mohapel P; Araujo IM; Popovic N; Li JY; Brundin P; Petersen A. 2005. Reduced hippocampal neurogenesis in R6/2 transgenic Huntington's disease mice. Neurobiol Dis 20(3):744-51. [PubMed: 15951191]  [MGI Ref ID J:104661]

Glynn D; Reim K; Brose N; Morton AJ. 2007. Depletion of Complexin II does not affect disease progression in a mouse model of Huntington's disease (HD); support for role for complexin II in behavioural pathology in a mouse model of HD. Brain Res Bull 72(2-3):108-20. [PubMed: 17352934]  [MGI Ref ID J:124474]

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]

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

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]

Hay DG; Sathasivam K; Tobaben S; Stahl B; Marber M; Mestril R; Mahal A; Smith DL; Woodman B; Bates GP. 2004. Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach. Hum Mol Genet 13(13):1389-405. [PubMed: 15115766]  [MGI Ref ID J:91194]

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]

Hemberger M; Himmelbauer H; Ruschmann J; Zeitz C; Fundele R. 2000. cDNA subtraction cloning reveals novel genes whose temporal and spatial expression indicates association with trophoblast invasion. Dev Biol 222(1):158-69. [PubMed: 10885754]  [MGI Ref ID J:62506]

Hickey MA; Gallant K; Gross GG; Levine MS; Chesselet MF. 2005. Early behavioral deficits in R6/2 mice suitable for use in preclinical drug testing. Neurobiol Dis 20(1):1-11. [PubMed: 16137562]  [MGI Ref ID J:102723]

Hickey MA; Reynolds GP; Morton AJ. 2002. The role of dopamine in motor symptoms in the R6/2 transgenic mouse model of Huntington's disease. J Neurochem 81(1):46-59. [PubMed: 12067237]  [MGI Ref ID J:75772]

Hockly E; Woodman B; Mahal A; Lewis CM; Bates G. 2003. Standardization and statistical approaches to therapeutic trials in the R6/2 mouse. Brain Res Bull 61(5):469-79. [PubMed: 13679245]  [MGI Ref ID J:102547]

Jenkins BG; Andreassen OA; Dedeoglu A; Leavitt B; Hayden M; Borchelt D; Ross CA; Ferrante RJ; Beal MF. 2005. Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease. J Neurochem 95(2):553-62. [PubMed: 16135087]  [MGI Ref ID J:129784]

Jin K; LaFevre-Bernt M; Sun Y; Chen S; Gafni J; Crippen D; Logvinova A; Ross CA; Greenberg DA; Ellerby LM. 2005. FGF-2 promotes neurogenesis and neuroprotection and prolongs survival in a transgenic mouse model of Huntington's disease. Proc Natl Acad Sci U S A 102(50):18189-94. [PubMed: 16326808]  [MGI Ref ID J:104367]

Johnson MA; Rajan V; Miller CE; Wightman RM. 2006. Dopamine release is severely compromised in the R6/2 mouse model of Huntington's disease. J Neurochem 97(3):737-46. [PubMed: 16573654]  [MGI Ref ID J:108545]

Johnson MA; Villanueva M; Haynes CL; Seipel AT; Buhler LA; Wightman RM. 2007. Catecholamine exocytosis is diminished in R6/2 Huntington's disease model mice. J Neurochem 103(5):2102-10. [PubMed: 17868298]  [MGI Ref ID J:128746]

Klapstein GJ; Fisher RS; Zanjani H; Cepeda C; Jokel ES; Chesselet MF; Levine MS. 2001. Electrophysiological and morphological changes in striatal spiny neurons in R6/2 Huntington's disease transgenic mice. J Neurophysiol 86(6):2667-77. [PubMed: 11731527]  [MGI Ref ID J:133106]

Klapstein GJ; Levine MS. 2005. Age-dependent biphasic changes in ischemic sensitivity in the striatum of Huntington's disease R6/2 transgenic mice. J Neurophysiol 93(2):758-65. [PubMed: 15371492]  [MGI Ref ID J:135135]

Kohl Z; Kandasamy M; Winner B; Aigner R; Gross C; Couillard-Despres S; Bogdahn U; Aigner L; Winkler J. 2007. Physical activity fails to rescue hippocampal neurogenesis deficits in the R6/2 mouse model of Huntington's disease. Brain Res 1155:24-33. [PubMed: 17512917]  [MGI Ref ID J:122499]

Kosinski CM; Cha JH; Young AB; Mangiarini L; Bates G; Schiefer J; Schwarz M. 1999. Intranuclear inclusions in subtypes of striatal neurons in Huntington's disease transgenic mice Neuroreport 10(18):3891-6. [PubMed: 10716229]  [MGI Ref ID J:61276]

Kung VW; Hassam R; Morton AJ; Jones S. 2007. Dopamine-dependent long term potentiation in the dorsal striatum is reduced in the R6/2 mouse model of Huntington's disease. Neuroscience 146(4):1571-80. [PubMed: 17478055]  [MGI Ref ID J:122067]

Kusakabe M; Mangiarini L; Laywell ED; Bates GP; Yoshiki A; Hiraiwa N; Inoue J; Steindler DA. 2001. Loss of cortical and thalamic neuronal tenascin-C expression in a transgenic mouse expressing exon 1 of the human Huntington disease gene. J Comp Neurol 430(4):485-500. [PubMed: 11169482]  [MGI Ref ID J:119265]

Levine MS; Klapstein GJ; Koppel A; Gruen E; Cepeda C; Vargas ME; Jokel ES; Carpenter EM; Zanjani H; Hurst RS; Efstratiadis A; Zeitlin S; Chesselet MF. 1999. Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease. J Neurosci Res 58(4):515-32. [PubMed: 10533044]  [MGI Ref ID J:76018]

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]

Li SH; Yu ZX; Li CL; Nguyen HP; Zhou YX; Deng C; Li XJ. 2003. Lack of huntingtin-associated protein-1 causes neuronal death resembling hypothalamic degeneration in Huntington's disease. J Neurosci 23(17):6956-64. [PubMed: 12890790]  [MGI Ref ID J:84682]

Lievens JC; Woodman B; Mahal A; Bates GP. 2002. Abnormal phosphorylation of synapsin I predicts a neuronal transmission impairment in the R6/2 Huntington's disease transgenic mice. Mol Cell Neurosci 20(4):638-48. [PubMed: 12213445]  [MGI Ref ID J:128200]

Luthi-Carter R; Hanson SA; Strand AD; Bergstrom DA; Chun W; Peters NL; Woods AM; Chan EY; Kooperberg C; Krainc D; Young AB; Tapscott SJ; Olson JM. 2002. Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain. Hum Mol Genet 11(17):1911-26. [PubMed: 12165554]  [MGI Ref ID J:78591]

Luthi-Carter R; Strand A; Peters NL; Solano SM; Hollingsworth ZR; Menon AS; Frey AS; Spektor BS; Penney EB; Schilling G; Ross CA; Borchelt DR; Tapscott SJ; Young AB; Cha JH; Olson JM. 2000. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Hum Mol Genet 9(9):1259-71. [PubMed: 10814708]  [MGI Ref ID J:62544]

Ma TC; Buescher JL; Oatis B; Funk JA; Nash AJ; Carrier RL; Hoyt KR. 2007. Metformin therapy in a transgenic mouse model of Huntington's disease. Neurosci Lett 411(2):98-103. [PubMed: 17110029]  [MGI Ref ID J:119010]

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]

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]

Meade CA; Deng YP; Fusco FR; Del Mar N; Hersch S; Goldowitz D; Reiner A. 2002. Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice. J Comp Neurol 449(3):241-69. [PubMed: 12115678]  [MGI Ref ID J:77468]

Menalled L; Zanjani H; MacKenzie L; Koppel A; Carpenter E; Zeitlin S; Chesselet MF. 2000. Decrease in striatal enkephalin mRNA in mouse models of Huntington's disease. Exp Neurol 162(2):328-42. [PubMed: 10739639]  [MGI Ref ID J:61687]

Meton I; Caseras A; Mediavilla D; Fernandez F; Baanante IV. 1999. Molecular cloning of a cDNA encoding 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase from liver of Sparus aurata: nutritional regulation of enzyme expression. Biochim Biophys Acta 1444(2):153-65. [PubMed: 10023046]  [MGI Ref ID J:53376]

Mihm MJ; Amann DM; Schanbacher BL; Altschuld RA; Bauer JA; Hoyt KR. 2007. Cardiac dysfunction in the R6/2 mousse model of Huntington's disease Neurobiol Dis 25(2):297-308. [PubMed: 17126554]  [MGI Ref ID J:116660]

Miller BR; Dorner JL; Shou M; Sari Y; Barton SJ; Sengelaub DR; Kennedy RT; Rebec GV. 2008. Up-regulation of GLT1 expression increases glutamate uptake and attenuates the Huntington's disease phenotype in the R6/2 mouse. Neuroscience 153(1):329-37. [PubMed: 18353560]  [MGI Ref ID J:136086]

Morton AJ; Edwardson JM. 2001. Progressive depletion of complexin II in a transgenic mouse model of Huntington's disease. J Neurochem 76(1):166-72. [PubMed: 11145989]  [MGI Ref ID J:67773]

Morton AJ; Leavens W. 2000. Mice transgenic for the human Huntington's disease mutation have reduced sensitivity to kainic acid toxicity. Brain Res Bull 52(1):51-9. [PubMed: 10779703]  [MGI Ref ID J:127958]

Morton AJ; Wood NI; Hastings MH; Hurelbrink C; Barker RA; Maywood ES. 2005. Disintegration of the sleep-wake cycle and circadian timing in Huntington's disease. J Neurosci 25(1):157-63. [PubMed: 15634777]  [MGI Ref ID J:96715]

Nagaoka U; Kim K; Jana NR; Doi H; Maruyama M; Mitsui K; Oyama F; Nukina N. 2004. Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions. J Neurochem 91(1):57-68. [PubMed: 15379887]  [MGI Ref ID J:93278]

Pallier PN; Maywood ES; Zheng Z; Chesham JE; Inyushkin AN; Dyball R; Hastings MH; Morton AJ. 2007. Pharmacological imposition of sleep slows cognitive decline and reverses dysregulation of circadian gene expression in a transgenic mouse model of Huntington's disease. J Neurosci 27(29):7869-78. [PubMed: 17634381]  [MGI Ref ID J:123314]

Papalexi E; Persson A; Bjorkqvist M; Petersen A; Woodman B; Bates GP; Sundler F; Mulder H; Brundin P; Popovic N. 2005. Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease. Eur J Neurosci 22(6):1541-6. [PubMed: 16190907]  [MGI Ref ID J:101557]

Petersen A; Gil J; Maat-Schieman ML; Bjorkqvist M; Tanila H; Araujo IM; Smith R; Popovic N; Wierup N; Norlen P; Li JY; Roos RA; Sundler F; Mulder H; Brundin P. 2005. Orexin loss in Huntington's disease. Hum Mol Genet 14(1):39-47. [PubMed: 15525658]  [MGI Ref ID J:136441]

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]

Petrasch-Parwez E; Habbes HW; Weickert S; Lobbecke-Schumacher M; Striedinger K; Wieczorek S; Dermietzel R; Epplen JT. 2004. Fine-structural analysis and connexin expression in the retina of a transgenic model of Huntington's disease. J Comp Neurol 479(2):181-97. [PubMed: 15452853]  [MGI Ref ID J:135880]

Phillips W; Jennifer Morton A; Barker RA. 2006. Limbic neurogenesis/plasticity in the R6/2 mouse model of Huntington's disease. Neuroreport 17(15):1623-1627. [PubMed: 17001281]  [MGI Ref ID J:114529]

Phillips W; Morton AJ; Barker RA. 2005. Abnormalities of neurogenesis in the R6/2 mouse model of Huntington's disease are attributable to the in vivo microenvironment. J Neurosci 25(50):11564-76. [PubMed: 16354914]  [MGI Ref ID J:128878]

Picconi B; Passino E; Sgobio C; Bonsi P; Barone I; Ghiglieri V; Pisani A; Bernardi G; Ammassari-Teule M; Calabresi P. 2006. Plastic and behavioral abnormalities in experimental Huntington's disease: a crucial role for cholinergic interneurons. Neurobiol Dis 22(1):143-52. [PubMed: 16326108]  [MGI Ref ID J:108042]

Qiu Z; Norflus F; Singh B; Swindell MK; Buzescu R; Bejarano M; Chopra R; Zucker B; Benn CL; DiRocco DP; Cha JH; Ferrante RJ; Hersch SM. 2006. Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotective. J Biol Chem 281(24):16672-80. [PubMed: 16595660]  [MGI Ref ID J:113725]

Rebec GV; Barton SJ; Ennis MD. 2002. Dysregulation of ascorbate release in the striatum of behaving mice expressing the Huntington's disease gene. J Neurosci 22(2):RC202. [PubMed: 11784814]  [MGI Ref ID J:79994]

Rebec GV; Barton SJ; Marseilles AM; Collins K. 2003. Ascorbate treatment attenuates the Huntington behavioral phenotype in mice. Neuroreport 14(9):1263-5. [PubMed: 12824772]  [MGI Ref ID J:89711]

Rebec GV; Conroy SK; Barton SJ. 2006. Hyperactive striatal neurons in symptomatic Huntington R6/2 mice: Variations with behavioral state and repeated ascorbate treatment. Neuroscience 137(1):327-36. [PubMed: 16257492]  [MGI Ref ID J:104610]

Ribchester RR; Thomson D; Wood NI; Hinks T; Gillingwater TH; Wishart TM; Court FA; Morton AJ. 2004. Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington's disease mutation. Eur J Neurosci 20(11):3092-114. [PubMed: 15579164]  [MGI Ref ID J:101274]

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]

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]

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]

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]

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]

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]

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]

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]

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]

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]

Yohrling GJ th; 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]

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]

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]

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]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX1

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
Diet Information LabDiet® 5K52/5K67

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Pricing for USA, Canada and Mexico shipping destinations View International pricing
Weeks of AgePrice (US dollars $)GenderGenotypes Provided
5 weeks $209.90Female or MaleHemizygous for Tg(HDexon1)62Gpb
6 weeks $213.80Female or MaleHemizygous for Tg(HDexon1)62Gpb
7 weeks $217.70Female or MaleHemizygous for Tg(HDexon1)62Gpb
Pairs /Price (US dollars $)Pair Genotype
$255.10Hemizygous for Tg(HDexon1)62Gpb (ovary transplant) x B6CBAF1/J (100011)

Additional Supply Details

Supply Notes
  • Shipped at a specific age in weeks. Mice at a precise age in days, littermates and retired breeders are also available.

Pricing for International shipping destinations View USA Canada and Mexico pricing
Weeks of AgePrice (US dollars $)GenderGenotypes Provided
5 weeks $272.90Female or MaleHemizygous for Tg(HDexon1)62Gpb
6 weeks $278.00Female or MaleHemizygous for Tg(HDexon1)62Gpb
7 weeks $283.10Female or MaleHemizygous for Tg(HDexon1)62Gpb
Pairs /Price (US dollars $)Pair Genotype
$331.70Hemizygous for Tg(HDexon1)62Gpb (ovary transplant) x B6CBAF1/J (100011)

Additional Supply Details

Supply Notes
  • 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.

Supply Details

Standard SupplyLevel 4. Up to 10 mice. Larger quantities or custom orders arranged upon request. Expected delivery up to one to three months.
Supply Notes
  • Strains that must be genotyped are not available until five to seven weeks of age.
  • This strain is a model for Huntington's Disease and as such becomes too ill to ship after seven weeks of age.
  • Genomic DNA is available for this strain from the Mouse DNA Resource.
Important NoteJanuary 2007: alteration in strain name and phenotype. Please see Strain Description for additional information.

Control Information

  Control
   Noncarrier
 
  Considerations for Choosing Controls
  USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
  International - Control Pricing Information for Genetically Engineered Mutant Strains.

General Terms and Conditions


See Terms of Use


The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
Ordering and Purchasing Information

      Purchasing Information
      JAX® Mice Orders
      Surgical Services

Contact Information
Orders & Technical Support
Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


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

Contact information

General inquiries

Contracts Administration

phone:207-288-6470
fax:207-288-6655

JAX® Mice & Services Conditions of Use

“Each recipient institution, including its employees and other researchers under its control (RECIPIENT), of mice or services using mice from The Jackson Laboratory (TJL) agrees that such mice, descendants of those mice derived by inbreeding or crossbreeding, including unmodified derivatives of those mice or their descendants (“MICE”) shall not be: (i) used for any purpose other than the internal research of the RECIPIENT, (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 with respect to MICE. Acceptance of MICE from TJL shall be deemed agreement by RECIPIENT to these conditions, and departure from these conditions requires The Jackson Laboratory’s prior written authorization.”

No Warranty

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

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

No Liability

In no event shall The Jackson Laboratory, 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 The Jackson Laboratory, its agents or employees. In purchasing or receiving MICE, products or services from The Jackson Laboratory, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges The Jackson Laboratory from all such causes of action or damages, and further agrees to defend and indemnify The Jackson Laboratory from any costs or damages arising out of any third party claims.

MICE and biological materials 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 The Jackson Laboratory’s MICE, products and services. In addition, special terms and conditions of sale of certain MICE, products and services may be set forth separately in The Jackson Laboratory 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 The Jackson Laboratory, 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 The Jackson Laboratory, 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 services by The Jackson Laboratory.


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