Article
N-terminal Huntingtin Knock-In Mice: Implications of Removing the N-terminal Region of Huntingtin for Therapy.
PLoS genetics - 1 May 2016
Liu Xudong, Wang Chuan-En, Hong Yan, Zhao Ting, Wang Guohao, Gaertig Marta A, Sun Miao, Li Shihua, Li Xiao-Jiang
Abstract excerpt
The Huntington's disease (HD) protein, huntingtin (HTT), is a large protein consisting of 3144 amino acids and has conserved N-terminal sequences that are followed by a polyglutamine (polyQ) repeat. Loss of Htt is known to cause embryonic lethality in mice, whereas polyQ expansion leads to adult neuronal degeneration. Whether N-terminal HTT is essential for neuronal development or contributes only to late-onset...
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