These HD100 mice express a transgene containing the initial N-terminal third of the mutant human huntingtin gene (IT15), and exhibit increased levels of nuclear and cytoplasmic huntingtin and dysmorphic dendrites in the striatum and cortex.
Neil Aronin, University of Massachusetts Medical School
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence, Humanized sequence) |
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.
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.
Expressed Gene | HTT, huntingtin, human |
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Site of Expression |
Allele Name | transgene insertion 63, Neil Aronin |
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Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | HD100L63; Tg100; TgCAG100 |
Gene Symbol and Name | Tg(HD)63Aron, transgene insertion 63, Neil Aronin |
Gene Synonym(s) | |
Promoter | Eno2, enolase 2, gamma, neuronal, rat |
Expressed Gene | HTT, huntingtin, human |
Strain of Origin | (C57BL/6 x SJL)F1 |
Chromosome | UN |
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 subcellular neuropathology 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. |
Mutations Made By | Neil Aronin, University of Massachusetts Medical School |
This 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
When using the HD100 mouse strain in a publication, please cite the originating article(s) and include JAX stock #004360 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Hemizygous or Non carrierfor Tg(HD)63Aron |
Frozen Mouse Embryo | B6;SJL-Tg(HD)63Aron/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6;SJL-Tg(HD)63Aron/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6;SJL-Tg(HD)63Aron/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | B6;SJL-Tg(HD)63Aron/J Frozen Embryo | $3373.50 |
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