Article
Disruption of the nuclear membrane by perinuclear inclusions of mutant huntingtin causes cell-cycle re-entry and striatal cell death in mouse and cell models of Huntington's disease.
Human molecular genetics - 15 Mar 2015
Liu Kuan-Yu, Shyu Yu-Chiau, Barbaro Brett A, Lin Yuan-Ta, Chern Yijuang, Thompson Leslie Michels, James Shen Che-Kun, Marsh J Lawrence
Abstract excerpt
Accumulation of N-terminal fragments of mutant huntingtin (mHTT) in the cytoplasm, nuclei and axons of neurons is a hallmark of Huntington's disease (HD), although how these fragments negatively impact neurons remains unclear. We followed the distribution of mHTT in the striata of transgenic R6/2-J2 HD mice as their motor function declined. The fraction of cells with diffuse, perinuclear or intranuclear mHTT...
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