Tg(HD)63Aron
Transgene Detail

Summary

• Symbol: Tg(HD)63Aron
• Name: transgene insertion 63, Neil Aronin
• MGI ID: MGI:3057174
• Synonyms: HD100L63, Tg100, TgCAG100
• Transgene: Tg(HD)63Aron Location: unknown
• Alliance: Tg(HD)63Aron page

Transgene origin

• Strain of Origin: (C57BL/6 x SJL)F1

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(HD)63Aron expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  HTT (3064)

  Htt	(MGI:96067)

• Mutation details: 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. (J:72772)

Disease models

Expression

In Structures Affected by this Mutation:

2 anatomical structure(s)

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 1 strain available Cell Lines: 0 lines available

Notes

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.

References

• 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)