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Review
. 2011 Feb 10;69(3):423-35.
doi: 10.1016/j.neuron.2010.12.035.

Huntington's disease: can mice lead the way to treatment?

Affiliations
Review

Huntington's disease: can mice lead the way to treatment?

Zachary R Crook et al. Neuron. .

Erratum in

  • Neuron. 2011 Mar 10;69(5):1038

Abstract

Mouse models for Huntington's Disease (HD) and HD patients demonstrate motor and behavioral dysfunctions, such as progressive loss of coordination and memory, and share similar transcriptional profiles and striatal neuron atrophy. Clear differences between the mouse and human diseases include almost complete striatal degeneration and rarity of intranuclear inclusions in HD, and the fact that mice expressing full-length mutant huntingtin do not demonstrate a shortened life span characteristic of HD. While no clinical interventions tested in mouse models to date have delayed disease progression, the mouse models provide an invaluable tool for both investigating the underlying pathogenic processes and developing new effective therapies. Inherent differences between humans and mice must be considered in the search for efficacious treatments for HD, but the striking similarities between human HD and mouse models support the view that these models are a biologically relevant system to support the identification and testing of potential clinical therapies.

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Figures

Figure 1
Figure 1
Timeline of behavioral and neuropathological symptoms in selected HD model mice. Strains are categorized by color: red (N-terminal transgenic), blue (full-length transgenic), and green (knock-in).

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