Strain Name:

B6;SJL-Tg(HD)63Aron/J

Stock Number:

004360

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Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Former Names B6;J-Tg(HD)63Aron/J    (Changed: 15-DEC-04 )
B6;SJL-Tg(HD)63Aron    (Changed: 15-DEC-04 )
Type Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
 
Donating Investigator Neil Aronin,   University of Massachusetts Medical School

Description
These transgenic mice express the initial N-terminal third of the mutant human huntingtin gene (IT15) under the direction of the rat neuron-specific enolase promoter. Expected transgene expression was confirmed by Northern blot, RT-PCR and Western blot analysis. Mice heterozygous for the transgene have a phenotype mimicking much of the morphological and subcellular neuropathology that occurs in the striatum and cortex in human Huntington's disease. Behavioral abnormalities are varible in onset and intensity, beginning between three to six months of age. Transgenic mice exhibit increased levels of nuclear and cytoplasmic huntingtin and dysmorphic dendrites in the striatum and cortex. Electron microscopic analysis of nuclear inclusions of cortical and striatal neurons detects granular and filamentous structures that appear to be similar to structures seen in brain affected by Huntington's disease. Cortical stimulation and N-methyl-D-aspartate (NMDA) receptor activation produces abnormal electrophysiological responses from striatal neurons.

Development
A transgenic construct containing bases 316-3210 of the human huntingtin cDNA sequence with a 100 CAG repeat insertion under the control of the rat neuron-specific enolase promoter and an SV40 polyadenylation signal, was introduced into oocyte pronuclei from C57BL/6 X SJL F1 hybrid mice. Transgenic mice were bred to B6;SJL mice.

Control Information

  Control
   Noncarrier
 
  Considerations for Choosing Controls

Related Strains

View Huntington's Disease Models     (29 strains)

View Strains carrying other alleles of Eno2     (12 strains)

View Strains carrying other alleles of HTT     (14 strains)

Additional Web Information

Visit our Huntington's Disease page for a full listing of Huntington's strains and research services.

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

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

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Tg(HD)63Aron/?

        involves: C57BL/6 * SJL
  • nervous system phenotype
  • abnormal cerebral cortex morphology
    • cytoplasmic huntingtin accumulation is prevalent in neurons of the frontal and cingulated cortices and occasionally present in the piriform and hippocampal cortices   (MGI Ref ID J:72772)
    • cortical pyramidal neurons exhibit retraction and disorientation of the apical dendrite, as well as dendritic abnormalities such as beading, small sharp bends, misalignment and bifurcation   (MGI Ref ID J:72772)
  • abnormal excitatory postsynaptic potential
    • stimulation of corpus callosum slices evoked smaller EPSPs in striatal neurons as compared to control   (MGI Ref ID J:72772)
    • response to stimulus in striatal neurons results in a rightward shift in the input-output curve (EPSP amplitude) as compared to control   (MGI Ref ID J:72772)
  • abnormal neuron morphology
    • one severely affected mouse exhibited shrunken neurons in the hippocampus and cerebellum   (MGI Ref ID J:72772)
    • intracytoplasmic vacuoles and plasma membrane blebs appear in cell bodies and dendrites of neurons with an accumulation of huntingtin   (MGI Ref ID J:72772)
    • abnormal dendrite morphology
      • cortical pyramidal neurons exhibit retraction and disorientation of the apical dendrite, as well as dendritic abnormalities such as beading, small sharp bends, misalignment and bifurcation   (MGI Ref ID J:72772)
      • some, but not all, mice exhibit dysmorphic dendrites in hippocampal pyramidal neurons   (MGI Ref ID J:72772)
      • one severely affected mouse had dysmorphic dendrites in cerebellar Purkinje cells   (MGI Ref ID J:72772)
      • dendrites in spiny neurons have significantly more curved endings (J-dendrites) and sharp bends (wavy dendrites) than control   (MGI Ref ID J:72772)
    • abnormal medium spiny neuron morphology
      • dendrites in spiny neurons have significantly more curved endings (J-dendrites) and sharp bends (wavy dendrites) than control   (MGI Ref ID J:72772)
    • neuronal intranuclear inclusions
      • intranuclear inclusions are detected in the cytoplasm of cortical neurons and to a lesser degree in striatal neurons   (MGI Ref ID J:72772)
      • in a sample of five mice, 27-70% of large striatal neurons that labeled for huntingtin had inclusions   (MGI Ref ID J:72772)
      • nuclear inclusions are composed of granules and filaments as determined by electron microscopy   (MGI Ref ID J:72772)
      • in 11 of 14 mice, inclusions appeared in a few cells in the hippocampus, substantia nigra, cerebellum and brainstem   (MGI Ref ID J:72772)
  • abnormal striatum morphology
    • htt staining is substantially increased in the cytoplasm of striatal neurons as compared to control   (MGI Ref ID J:72772)
    • abnormal medium spiny neuron morphology
      • dendrites in spiny neurons have significantly more curved endings (J-dendrites) and sharp bends (wavy dendrites) than control   (MGI Ref ID J:72772)
  • enhanced NMDA-mediated synaptic currents
    • one population of mutant neurons (most affected) exhibit higher peak currents and current densities when induced by NMDA as well as an increase in calcium ion influx   (MGI Ref ID J:72772)
  • behavior/neurological phenotype
  • abnormal gait
    • some mice exhibit an abnormal gait which could include wide-based gait, walking with an arched posture and slow gait   (MGI Ref ID J:72772)
  • abnormal locomotor activation
    • some mice exhibit an alteration in activity that is scored as hypo- or hyperactivity   (MGI Ref ID J:72772)
  • impaired coordination
    • in rotarod performance   (MGI Ref ID J:72772)
  • limb grasping
    • most mice exhibit clasping in the tail suspension test   (MGI Ref ID J:72772)
View Research Applications

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

HTT related

Developmental Biology Research
Neurodevelopmental Defects

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

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(HD)63Aron
Allele Name transgene insertion 63, Neil Aronin
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) HD100L63; Tg100; TgCAG100;
Mutation Made By Neil Aronin,   University of Massachusetts Medical School
Strain of Origin(C57BL/6 x SJL)F1
Expressed Gene HTT, huntingtin, human
Promoter Eno2, enolase 2, gamma, neuronal, rat
General Note Transgenic mice express the initial N-terminal third of the mutant human huntingtin gene (IT15) under the direction of the rat neuron-specific enolase promoter. The phenotype of hemizygous transgenic mice mimicks much of the morphological and subcellularneuropathology that occurs in the striatum and cortex in the human Huntington disease. Onset and intensity of behavioral abnormalities are variable and begin between 3 to 6 months of age.

Transgenic mice exhibit increased levels of nuclear and cytoplasmic huntingtin and dysmorphic dendrites in the striatum and cortex. Electron microscopic analysis of nuclear inclusions of cortical and striatal neurons detects granular and filamentous structures that appear to be similar to structures seen in human brain affected by Huntington's disease. Cortical stimulation and N-methyl-D-aspartate (NMDA) receptor activation produce abnormal electrophysiological responses from striatal neurons of transgenic mice.

Molecular Note The transgene contains bases 316-3210 of the human huntingtin cDNA sequence with a 100 CAG repeat insertion, under the control of the rat neuron-specific enolase promoter and an SV40 polyadenylation signal. Northern blot, RT-PCR, and Western blot analyses detected expression in neural cells. [MGI Ref ID J:72772]
 
 

Genotyping

Genotyping Information

Genotyping Protocols

Tg(HD)63Aron, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Laforet GA; Sapp E; Chase K; McIntyre C; Boyce FM; Campbell M; Cadigan BA; Warzecki L; Tagle DA; Reddy PH; Cepeda C; Calvert CR; Jokel ES; Klapstein GJ; Ariano MA; Levine MS; DiFiglia M; Aronin N. 2001. Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J Neurosci 21(23):9112-23. [PubMed: 11717344]  [MGI Ref ID J:72772]

Additional References

Tg(HD)63Aron related

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]

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

Health & husbandry

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Breeding & HusbandryThis strain originated on a B6;SJL background and has been backcrossed for 10 generations on the B6;SJL background. Coat color expected from breeding:Black and Agouti

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2140.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

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

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2782.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

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

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

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

Control Information

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

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