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. 2007 Apr 30;72(2-3):98-102.
doi: 10.1016/j.brainresbull.2006.10.015. Epub 2006 Nov 13.

Progressive CAG expansion in the brain of a novel R6/1-89Q mouse model of Huntington's disease with delayed phenotypic onset

Sarat C Vatsavayai  1 , Glenn M Dallérac, Austen J Milnerwood, Damian M Cummings, Payam Rezaie, Kerry P S J Murphy, Mark C Hirst
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Progressive CAG expansion in the brain of a novel R6/1-89Q mouse model of Huntington's disease with delayed phenotypic onset

Sarat C Vatsavayai et al. Brain Res Bull. .

Abstract

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.

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