Exp Neurobiol 2017; 26(6): 321-328
Published online December 31, 2017
© The Korean Society for Brain and Neural Sciences
Dong-Kyu Kim1, Kyu-Won Cho1, Woo Jung Ahn1, Dayana Perez-Acuña1,Hyunsu Jeong1,2, He-Jin Lee3,4 and Seung-Jae Lee1*
1Department of Medicine and Biomedical Sciences and Neuroscience Research Institute, Seoul National University College ofMedicine, Seoul 03080, 2Department of Psychology, Seoul National University, Seoul 08826, 3Department of Anatomy, School of Medicine, Konkuk University, Seoul 05029, 4IBST, Konkuk University, Seoul 05029, Korea
Correspondence to: *To whom correspondence should be addressed.
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Huntington disease (HD) is an inherited neurodegenerative disorder characterized by motor and cognitive dysfunction caused by expansion of polyglutamine (polyQ) repeat in exon 1 of huntingtin (HTT). In patients, the number of glutamine residues in polyQ tracts are over 35, and it is correlated with age of onset, severity, and disease progression. Expansion of polyQ increases the propensity for HTT protein aggregation, process known to be implicated in neurodegeneration. These pathological aggregates can be transmitted from neuron to another neuron, and this process may explain the pathological spreading of polyQ aggregates. Here, we developed an