Vatsavayai, Sarat C.; Dallerac, Glenn M.; Milnerwood, Austen J.; Cummings, Damian M.; Rezaie, Payam; Murphy, Kerry P.S.J. and Hirst, Mark C.
|DOI (Digital Object Identifier) Link:||http://dx.doi.org/10.1016/j.brainresbull.2006.10.015|
|Google Scholar:||Look up in Google Scholar|
Transgenic models representing Huntington's disease (HD) have proved useful for understanding the cascade of molecular events leading to the disease. We report an initial characterisation of a novel transgenic mouse model derived from a spontaneous truncation event within the R6/1 transgene. The transgene is widely expressed, carries 89 CAG repeats and the animals exhibit a significantly milder neurological phenotype with delayed onset compared to R6/1. Moreover, we report evidence of progressive somatic CAG expansions in the brain starting at an early age before an overt phenotype has developed. This novel line shares a common genetic ancestry with R6/1, differing only in CAG repeat number, and therefore, provides an additional tool with which to examine early molecular and neurophysiological changes in HD.
|Item Type:||Journal Article|
|Academic Unit/Department:||Science > Life, Health and Chemical Sciences
|Interdisciplinary Research Centre:||Biomedical Research Network (BRN)|
|Depositing User:||Astrid Peterkin|
|Date Deposited:||25 Apr 2007|
|Last Modified:||14 Jan 2016 16:31|
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