JAX Mouse Strain Datasheet - 006494

B6CBA-Tg(HDexon1)62Gpb/3J

Stock No:: 006494 |; B6CBA-R6/2 (CAG 120 +/- 5)

Transgenic

Available Now

Overview

JAXTag^TM

Also Known As: B6CBA-R6/2 (CAG 120 +/- 5)

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.

Donating Investigator

Gillian P Bates, University College London, Institute of Neurology

Genetic overview

Genetic Background	Generation
	N58 (14-AUG-14)

Tg(HDexon1)62Gpb

Allele Type
Transgenic (Humanized sequence, Inserted expressed sequence)

View Genetics

Research Applications

Cardiovascular Research
Diabetes and Obesity Research
Developmental Biology Research
Neurobiology Research

View All Research Applications

Base Price

Starting at:

$307.62 Domestic price for female 3-week

$354.60 Domestic price for breeder pair

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Details

Important Note

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

Detailed 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)

The HDexon1 transgene functions as single copy insertion. Sequence analysis identified its insertion site within an intron of the predicted gene Gm12695 on mouse chromosome 4 (chr4:96,409,585-96,414,930 [assembly NCBI 37/mus musuculus 9)], and the transgene is flanked by two rearranged sequences that do not contain the full exon 1 encoding DNA (Cowin et al. 2011 PLoS ONE 6(12):e28409). Additionally, a segment of Gram-positive bacterial sequence (likely originating from cloning vector contamination) is inserted just upstream of the HTT promoter that drives the expression of the intact copy. Transgene insertion also resulted in a 5.4 kbp deletion of mouse chromosomal DNA near the integration site (Chiang et al. 2012 Nat Genet. 44(4):390-7). As of January 2017, the function of predicted gene Gm12695 is unknown. It is normally expressed at negligible levels in mouse brain. The transgene insertion (in antisense orientation to Gm12695 transcription) results in increased cortical expression of a partial Gm12695 fragment (exons 8-11) - and this transcript is shown to have significant expression among the extensive network of differentially expressed genes associated with the R6/2 model, including those regulating synaptic transmission, cell signaling and transcription (Jacobsen et al. 2017 Sci Rep. 7:41120).

This strain ships with a JAXTag^TM affixed. Learn more about JAXTag^TM.

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.

Expression Data

Expressed Gene	HTT, huntingtin, human
Site of Expression

Control Suggestions

Noncarrier

Additional Information

Considerations for Choosing Controls

Selected References

Cummings DM; Alaghband Y; Hickey MA; Joshi PR; Hong SC; Zhu C; Ando TK; Andre VM; Cepeda C; Watson JB; Levine MS. 2012. A critical window of CAG repeat-length correlates with phenotype severity in the R6/2 mouse model of Huntington's disease. J Neurophysiol 107(2):677-91. PubMed: 22072510 MGI: J:229736
Grima JC; Daigle JG; Arbez N; Cunningham KC; Zhang K; Ochaba J; Geater C; Morozko E; Stocksdale J; Glatzer JC; Pham JT; Ahmed I; Peng Q; Wadhwa H; Pletnikova O; Troncoso JC; Duan W; Snyder SH; Ranum LP; Thompson LM; Lloyd TE; Ross CA; Rothstein JD. 2017. Mutant Huntingtin Disrupts the Nuclear Pore Complex. Neuron 94(1):93-107.e6. PubMed: 28384479 MGI: J:240166
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: 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: J:185262

View All References

Genetics

Tg(HDexon1)62Gpb

Allele Symbol: Tg(HDexon1)62Gpb

Allele Name	transgene insertion 62, Gillian Bates
Allele Type	Transgenic (Humanized sequence, Inserted expressed sequence)
Allele Synonym(s)	R6/2; R6/2B; Tg(HDexon1)62nGpb
Gene Symbol and Name	Tg(HDexon1)62Gpb, transgene insertion 62, Gillian Bates
Gene Synonym(s)	R6/2; Tg(HDexon1)62nGpb
Promoter	HTT, huntingtin, human
Expressed Gene	HTT, huntingtin, human
Strain of Origin	CBA x C57BL/6
Chromosome	4
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, and 262 bp of intron 1. 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, transgenic 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.
Mutations Made By	Gillian Bates, University College London, Institute of Neurology

Disease/Phenotype

Disease Terms

Research Areas By Genotype

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

Genotype: 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

Mammalian Phenotype Terms by Genotype

Genotype: 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

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

abnormal striatum morphology
- immunoreactive htt is detected in the striatum at 4.5 weeks

decreased brain weight
- reduction in brain weight by 5 weeks

neuronal intranuclear inclusions
- 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 appear in the cerebral cortex before they can be detected in the striatum
- inclusions are granular with occasional filamentous structures around the periphery; they are larger than the nucleolus and occupy 1% of nuclear volume
- 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 are ubiquitinated by 5-6 weeks and can be detected by ultrastructural analysis by 8 weeks

growth/size/body region phenotype

decreased body weight
- progressive loss of body weight

Genotype: 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

decreased grip strength
- progressive deterioration of grip strength especially in males between 10-14 weeks of age

decreased startle reflex
- beginning at 4 weeks of age

decreased vertical activity
- mice exhibit a progressive decrease in climbing activity starting at 4 weeks of age
- 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

hypoactivity
- in the dark phase both sexes are hypoactive beginning at 12 weeks of age
- in the light phase females are less active at 8 and 14 weeks of age
- in the light phase males are less active starting at 6 weeks of age
- mice are hypoactive in the open field test in both the light and dark phases of the light cycle
- progressive hypoactivity is observed in the center of the open field beginning at 4 weeks of age

impaired coordination
- latency time decreases with age
- mice exhibit a reduced latency to fall on rotarod test beginning at 4 weeks of age as compared to wild-type

increased anxiety-related response
- increased preference for dark in dark/light choice test at 12 and 14 weeks of age

short stride length
- decreased stride length (ipsilateral) observed at 8 weeks of age
- shorter splay length (contralateral) than controls

growth/size/body region phenotype

decreased body weight
- decrease in body weight is observed by 7 weeks in both genders

mortality/aging

premature death
- all mice die within 12 weeks of the first death
- median survival of 113 days

nervous system phenotype

decreased prepulse inhibition
- observed after 8 weeks of age

Genotype: Tg(HDexon1)62Gpb/0
involves: C57BL/6J * CBA/J

mortality/aging

premature death
- mice can die suddenly
- usually between 10 to 13 weeks of age

nervous system phenotype

decreased brain size
- average of 19% smaller than wildtype

seizures
- handling induced seizures that can be several minutes duration

behavior/neurological phenotype

abnormal vocalization
- 2 distinct characteristic vocalizations observed

impaired balance
- mice lose balance when sitting on hind limbs, turning and grooming their backs

limb grasping
- dyskinesia of limbs when suspended by the tail

seizures
- handling induced seizures that can be several minutes duration

stereotypic behavior
- 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
- involuntary jerky shudders
- progressive resting tremor in limbs, trunk and head

endocrine/exocrine gland phenotype

abnormal pancreatic alpha cell morphology
- islets exhibit huntingtin inclusions in 24% of cells by 12 weeks of age

abnormal pancreatic beta cell morphology
- islets are hypotrophic by 12 weeks, BrdU incorporation is reduced 6-fold
- islets are smaller in size by 12 weeks
- islets contain few insulin secretory vesicles by 12 weeks
- islets exhibit huntingtin inclusions in 19% of cells by 7 weeks of age, by week 12 frequency is greater than 95%
- significant reduction in insulin and somatostatin content by 12 weeks

abnormal pancreatic delta cell morphology
- islets exhibit huntingtin inclusions in 6% of cells by 12 weeks of age

decreased pancreatic beta cell number
- by 12 weeks, no signs of apoptosis or necrosis are observed

small ovary

small seminal vesicle
- small seminal ducts and gland size

small testis

growth/size/body region phenotype

decreased body weight
- body weight declines after 10 weeks

weight loss
- body weight is normal at weaning
- overall loss of muscle bulk observed
- with progression of phenotype up to 30% of body weight can be lost

homeostasis/metabolism phenotype

abnormal glucose homeostasis
- abnormal glucose homeostasis

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

impaired glucose tolerance
- identified at 11.5 weeks of age, insulin response absent

renal/urinary system phenotype

polyuria

reproductive system phenotype

abnormal female reproductive system morphology
- smaller size ovaries and uteri often observed

female infertility

small ovary

small seminal vesicle
- small seminal ducts and gland size

small testis

small uterus

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

Genotype: Tg(HDexon1)62Gpb/0
B6.Cg-Tg(HDexon1)62Gpb/240

behavior/neurological phenotype

abnormal gait
- narrower base (separation between legs) than controls

decreased grip strength
- progressive deterioration of grip strength especially in males at 14 weeks of age

decreased startle reflex
- observed at 6 and 14 weeks of age in both genders

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
- rearing is similar to controls in dark phase

hypoactivity
- in dark phase, hypoactivity is significant at 6 weeks of age in both genders
- mice cover less distance in center starting at 12 weeks in light phase and 6 weeks in dark phase
- 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

impaired coordination
- mice exhibit a progressive impairment on the rotarod test beginning at 8 weeks of age

increased anxiety-related response
- increased preference for dark in dark/light choice test at 14 weeks of age

short stride length
- shorter splay length (contralateral) than controls at 14 weeks of age
- stride length (ipsilateral) is similar to controls

growth/size/body region phenotype

decreased body weight
- body weight decreases by 10 weeks of age in females and 9 weeks in males

mortality/aging

premature death
- all mice die within 9 weeks of the first death
- median survival of 141 days for females and 179 days for males

nervous system phenotype

decreased prepulse inhibition
- observed at 14 weeks of age, especially in females

Genotype: Tg(HDexon1)62Gpb/0
involves: C57BL/6 * CBA/J

vision/eye phenotype

abnormal photoreceptor inner segment morphology
- inner segment is reduced in thickness at 10 weeks of age

abnormal photoreceptor outer segment morphology
- outer segment is reduced in thickness at 10 weeks of age

abnormal retina morphology
- retinal dysplasia covers more than 50% of the entire retina

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

abnormal retinal outer plexiform layer morphology
- however, no inner plexiform layer or ganglion cell layer abnormalities
- misplaced photoreceptor nuclei are seen in the outer plexiform layer at 10 weeks of age

abnormal retinal photoreceptor morphology
- misplaced photoreceptor nuclei are seen in the segment layers at 10 weeks of age

disorganized photoreceptor inner segment
- seen at 10 weeks of age

disorganized photoreceptor outer segment
- seen at 10 weeks of age

retinal photoreceptor degeneration

nervous system phenotype

abnormal photoreceptor inner segment morphology
- inner segment is reduced in thickness at 10 weeks of age

abnormal photoreceptor outer segment morphology
- outer segment is reduced in thickness at 10 weeks of age

abnormal retinal photoreceptor morphology
- misplaced photoreceptor nuclei are seen in the segment layers at 10 weeks of age

disorganized photoreceptor inner segment
- seen at 10 weeks of age

disorganized photoreceptor outer segment
- seen at 10 weeks of age

neuronal intranuclear inclusions
- nuclear inclusions are seen in the retina

retinal photoreceptor degeneration

Genotype: Tg(HDexon1)62Gpb/0
involves: C57BL/6 * C57BL/6JOlaHsd * CBA * CBA/CaOlaHsd

behavior/neurological phenotype

decreased grip strength

impaired coordination

nervous system phenotype

decreased brain weight

References

Cummings DM; Alaghband Y; Hickey MA; Joshi PR; Hong SC; Zhu C; Ando TK; Andre VM; Cepeda C; Watson JB; Levine MS. 2012. A critical window of CAG repeat-length correlates with phenotype severity in the R6/2 mouse model of Huntington's disease. J Neurophysiol 107(2):677-91. PubMed: 22072510 MGI: J:229736
Grima JC; Daigle JG; Arbez N; Cunningham KC; Zhang K; Ochaba J; Geater C; Morozko E; Stocksdale J; Glatzer JC; Pham JT; Ahmed I; Peng Q; Wadhwa H; Pletnikova O; Troncoso JC; Duan W; Snyder SH; Ranum LP; Thompson LM; Lloyd TE; Ross CA; Rothstein JD. 2017. Mutant Huntingtin Disrupts the Nuclear Pore Complex. Neuron 94(1):93-107.e6. PubMed: 28384479 MGI: J:240166
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: 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: J:185262

Additional - 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: J:144556
Acuna AI; Esparza M; Kramm C; Beltran FA; Parra AV; Cepeda C; Toro CA; Vidal RL; Hetz C; Concha II; Brauchi S; Levine MS; Castro MA. 2013. A failure in energy metabolism and antioxidant uptake precede symptoms of Huntington's disease in mice. Nat Commun 4:2917. PubMed: 24336051 MGI: J:226221
Adelfinger L; Turecek R; Ivankova K; Jensen AA; Moss SJ; Gassmann M; Bettler B. 2014. GABAB receptor phosphorylation regulates KCTD12-induced K(+) current desensitization. Biochem Pharmacol 91(3):369-79. PubMed: 25065880 MGI: J:215382
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: 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: 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: 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: 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: 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: J:183404
Bari M; Battista N; Valenza M; Mastrangelo N; Malaponti M; Catanzaro G; Centonze D; Finazzi-Agro A; Cattaneo E; Maccarrone M. 2013. In vitro and in vivo models of Huntington's disease show alterations in the endocannabinoid system. FEBS J 280(14):3376-88. PubMed: 23659592 MGI: J:214541
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: 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: 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: 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: 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: J:199086
Benraiss A; Wang S; Herrlinger S; Li X; Chandler-Militello D; Mauceri J; Burm HB; Toner M; Osipovitch M; Jim Xu Q; Ding F; Wang F; Kang N; Kang J; Curtin PC; Brunner D; Windrem MS; Munoz-Sanjuan I; Nedergaard M; Goldman SA. 2016. Human glia can both induce and rescue aspects of disease phenotype in Huntington disease. Nat Commun 7:11758. PubMed: 27273432 MGI: J:239898
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: 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: 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: 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: 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: J:138558
Blum D; Herrera F; Francelle L; Mendes T; Basquin M; Obriot H; Demeyer D; Sergeant N; Gerhardt E; Brouillet E; Buee L; Outeiro TF. 2015. Mutant huntingtin alters Tau phosphorylation and subcellular distribution. Hum Mol Genet 24(1):76-85. PubMed: 25143394 MGI: J:216514
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: 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: J:174136
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: J:73480
Bouchard J; Truong J; Bouchard K; Dunkelberger D; Desrayaud S; Moussaoui S; Tabrizi SJ; Stella N; Muchowski PJ. 2012. Cannabinoid receptor 2 signaling in peripheral immune cells modulates disease onset and severity in mouse models of Huntington's disease. J Neurosci 32(50):18259-68. PubMed: 23238740 MGI: J:192012
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: J:99425
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Also Known As: B6CBA-R6/2 (CAG 120 +/- 5)

Donating Investigator

Genetic overview

Research Applications

Base Price

Important Note

Detailed Description

Development

Expression Data

Control Suggestions

Additional Information

Selected References

Tg(HDexon1)62Gpb

Allele Symbol: Tg(HDexon1)62Gpb

Disease Terms

Research Areas By Genotype

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

Genotype: HTT related

Mammalian Phenotype Terms by Genotype

Genotype: Tg(HDexon1)62Gpb/0involves: C57BL/6 * CBA

nervous system phenotype

growth/size/body region phenotype

Genotype: Tg(HDexon1)62Gpb/0B6CBA-Tg(HDexon1)62Gpb/3J

behavior/neurological phenotype

growth/size/body region phenotype

mortality/aging

nervous system phenotype

Genotype: Tg(HDexon1)62Gpb/0involves: C57BL/6J * CBA/J

mortality/aging

nervous system phenotype

behavior/neurological phenotype

endocrine/exocrine gland phenotype

growth/size/body region phenotype

homeostasis/metabolism phenotype

renal/urinary system phenotype

reproductive system phenotype

Genotype: Tg(HDexon1)62Gpb/0B6.Cg-Tg(HDexon1)62Gpb/240

behavior/neurological phenotype

growth/size/body region phenotype

mortality/aging

nervous system phenotype

Genotype: Tg(HDexon1)62Gpb/0involves: C57BL/6 * CBA/J

vision/eye phenotype

nervous system phenotype

Genotype: Tg(HDexon1)62Gpb/0involves: C57BL/6 * C57BL/6JOlaHsd * CBA * CBA/CaOlaHsd

behavior/neurological phenotype

nervous system phenotype

References

Additional - Tg(HDexon1)62Gpb related

Genotyping Protocols

Dietary Information

Breeding Considerations

Appearance

Animal Health Reports

Live Mouse

Breeder Pair

Cryorecovery - Pricing

Progeny testing is not required

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Credit for PRODUCTS or SERVICES

Animal Care and Use for SERVICES

No Liability

Genotype: Tg(HDexon1)62Gpb/0
involves: C57BL/6 * CBA

Genotype: Tg(HDexon1)62Gpb/0
B6CBA-Tg(HDexon1)62Gpb/3J

Genotype: Tg(HDexon1)62Gpb/0
involves: C57BL/6J * CBA/J

Genotype: Tg(HDexon1)62Gpb/0
B6.Cg-Tg(HDexon1)62Gpb/240

Genotype: Tg(HDexon1)62Gpb/0
involves: C57BL/6 * CBA/J

Genotype: Tg(HDexon1)62Gpb/0
involves: C57BL/6 * C57BL/6JOlaHsd * CBA * CBA/CaOlaHsd