Huntington's Disease: Can Mice Lead the Way to Treatment?
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Housman_Huntington's disease.pdf
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507.27 KB
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Checksum (MD5)
08c2d6508a79c6ef9bd74ce558a9ef1e
Author(s)
Crook, Zachary Ryan
Housman, David E
Date Issued
February 2011
Journal
Neuron
Publisher
Elsevier
Citation
Crook, Zachary R., and David Housman. “Huntington s Disease: Can Mice Lead the Way to Treatment?” Neuron 69, no. 3 (February 2011): 423-435. Copyright © 2011 Elsevier Inc.
Version
Final published version
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 characteritstic 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.
MIT Department
Massachusetts Institute of Technology. Department of Biology
Koch Institute for Integrative Cancer Research at MIT
Terms of Use
Article is made available in accordance with the publisher's policy and may be subject to US copyright law. Please refer to the publisher's site for terms of use.
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DOI of Published Version
https://doi.org/10.1016/j.neuron.2010.12.035
