Article
Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease.
Human molecular genetics - 1 May 2015
Jin Jing, Peng Qi, Hou Zhipeng, Jiang Mali, Wang Xin, Langseth Abraham J, Tao Michael, Barker Peter B, Mori Susumu, Bergles Dwight E, Ross Christopher A, Detloff Peter J, Zhang Jiangyang, Duan Wenzhen
Abstract excerpt
White matter abnormalities have been reported in premanifest Huntington's disease (HD) subjects before overt striatal neuronal loss, but whether the white matter changes represent a necessary step towards further pathology and the underlying mechanism of these changes remains unknown. Here, we characterized a novel knock-in mouse model that expresses mouse HD gene homolog (Hdh) with extended CAG repeat- HdhQ250,...
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