Lessons Learned from the Transgenic Huntington's Disease Rats

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 therapie...

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Bibliographic Details
Main Authors: Rinske Vlamings, Dagmar H. Zeef, Marcus L. F. Janssen, Mayke Oosterloo, Frederic Schaper, Ali Jahanshahi, Yasin Temel
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Neural Plasticity
Online Access:http://dx.doi.org/10.1155/2012/682712
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Summary: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.
ISSN:2090-5904
1687-5443