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Transgene Detail
Symbol: Tg(HD)63Aron
Name: transgene insertion 63, Neil Aronin
MGI ID: MGI:3057174
Synonyms: HD100L63, Tg100, TgCAG100
Transgene: Tg(HD)63Aron  Location: unknown  
Strain of Origin:  (C57BL/6 x SJL)F1
Transgene Type:    Transgenic (Humanized sequence, Inserted expressed sequence)
Mutation:    Insertion
Tg(HD)63Aron expresses 1 gene
Mutation detailsThe 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. (J:72772)
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Carrying this Mutation:  Mouse Strains: 1 strain available      Cell Lines: 0 lines available
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.

Original:  J:72772 Laforet GA, et al., Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J Neurosci. 2001 Dec 1;21(23):9112-23
All:  3 reference(s)

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MGI 6.01
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