Article
N17 Modifies mutant Huntingtin nuclear pathogenesis and severity of disease in HD BAC transgenic mice.
Neuron - 18 Feb 2015
Gu Xiaofeng, Cantle Jeffrey P, Greiner Erin R, Lee C Y Daniel, Barth Albert M, Gao Fuying, Park Chang Sin, Zhang Zhiqiang, Sandoval-Miller Susana, Zhang Richard L, Diamond Marc, Mody Istvan, Coppola Giovanni, Yang X William
Abstract excerpt
The nucleus is a critical subcellular compartment for the pathogenesis of polyglutamine disorders, including Huntington's disease (HD). Recent studies suggest the first 17-amino-acid domain (N17) of mutant huntingtin (mHTT) mediates its nuclear exclusion in cultured cells. Here, we test whether N17 could be a molecular determinant of nuclear mHTT pathogenesis in vivo. BAC transgenic mice expressing mHTT lacking...
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