Article
Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 15 Nov 2001
Martín-Aparicio E, Yamamoto A, Hernández F, Hen R, Avila J, Lucas J J
Abstract excerpt
Neuronal intranuclear inclusions are a histopathological hallmark of Huntington's disease. Nevertheless, the precise mechanism by which they are formed and their relevance to neuronal cell death and/or dysfunction remains unclear. We recently generated a conditional mouse model of Huntington's disease (HD94) in which silencing expression of mutated huntingtin led to the disappearance of intranuclear aggregates...
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