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Article

Neuronal aggregates are associated with phenotypic onset in the R6/2 Huntington's disease transgenic mouse.

Behavioural brain research - 15 Apr 2012

Cowin Randi-Michelle, Roscic Ana, Bui Nghiem, Graham Deanna, Paganetti Paolo, Jankowsky Joanna L, Weiss Andreas, Paylor Richard

Abstract excerpt

Huntington's disease (HD) is caused by the expansion of the polyglutamine tract expressed in the huntingtin protein. Data from patients show a strong negative correlation between CAG repeat size and age of disease onset. Recent studies in mixed background C57×CBA R6/2 mice suggest the inverse correlation observed in the human disease may not be replicated in some animal models of HD. To further clarify the...

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PMID: 22306231
DOI: 10.1016/j.bbr.2011.12.045

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