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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

Vatsavayai, SC; Dallerac, GM; Milnerwood, AJ; Cummings, DM; Rezaie, P; Murphy, KPSJ; Hirst, MC; (2007) Progressive CAG expansion in the brain of a novel R6/1-89Q mouse model of Huntington's disease with delayed phenotypic onset. BRAIN RESEARCH BULLETIN , 72 (2-3) 98 - 102. 10.1016/j.brainresbull.2006.10.015.

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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. (c) 2006 Published by Elsevier Inc.

Type: Article
Title: Progressive CAG expansion in the brain of a novel R6/1-89Q mouse model of Huntington's disease with delayed phenotypic onset
Location: Manchester, ENGLAND
DOI: 10.1016/j.brainresbull.2006.10.015
Keywords: Huntingtin, transgenic, trinucleotide repeat, cortex, hippocampus, Huntington, TRINUCLEOTIDE REPEAT, WIDESPREAD EXPRESSION, GENE, MICE, LOCALIZATION, INSTABILITY, TISSUES, PRODUCT, LENGTH
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Life Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Life Sciences > Div of Biosciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Life Sciences > Div of Biosciences > Neuro, Physiology and Pharmacology
URI: http://discovery.ucl.ac.uk/id/eprint/1333294
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