Article
Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 11 Jun 2008
Gray Michelle, Shirasaki Dyna I, Cepeda Carlos, André Véronique M, Wilburn Brian, Lu Xiao-Hong, Tao Jifang, Yamazaki Irene, Li Shi-Hua, Sun Yi E, Li Xiao-Jiang, Levine Michael S, Yang X William
Abstract excerpt
To elucidate the pathogenic mechanisms in Huntington's disease (HD) elicited by expression of full-length human mutant huntingtin (fl-mhtt), a bacterial artificial chromosome (BAC)-mediated transgenic mouse model (BACHD) was developed expressing fl-mhtt with 97 glutamine repeats under the control of endogenous htt regulatory machinery on the BAC. BACHD mice exhibit progressive motor deficits, neuronal synaptic...
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