Article
Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation.
Human molecular genetics - 1 Apr 2011
Havel Lauren S, Wang Chuan-En, Wade Brandy, Huang Brenda, Li Shihua, Li Xiao-Jiang
Abstract excerpt
An expanded polyglutamine tract (>37 glutamines) in the N-terminal region of huntingtin (htt) causes htt to accumulate in the nucleus, leading to transcriptional dysregulation in Huntington disease (HD). In HD knock-in mice that express full-length mutant htt at the endogenous level, mutant htt preferentially accumulates in the nuclei of striatal neurons, which are affected most profoundly in HD. The mechanism...
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