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Neural Plasticity
Volume 2012, Article ID 682712, 8 pages
Review Article

Lessons Learned from the Transgenic Huntington's Disease Rats

1Department of Neuroscience, Maastricht University Medical Center, 6229 ER Maastricht, The Netherlands
2Department of Neurology, Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands
3Department of Neurosurgery, Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands

Received 13 February 2012; Revised 21 May 2012; Accepted 4 June 2012

Academic Editor: Emma Frost

Copyright © 2012 Rinske Vlamings et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Huntington's disease (HD) is a fatal inherited disorder leading to selective neurodegeneration and neuropsychiatric symptoms. Currently, there is no treatment to slow down or to stop the disease. There is also no therapy to effectively reduce the symptoms. In the investigation of novel therapies, different animal models of Huntington's disease, varying from insects to nonhuman primates, have been created and used. Few years ago, the first transgenic rat model of HD, carrying a truncated huntingtin cDNA fragment with 51 CAG repeats under control of the native rat huntingtin promoter, was introduced. We have been using this animal model in our research and review here our experience with the behavioural, neurophysiological, and histopathological phenotype of the transgenic Huntington's disease rats with relevant literature.