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USA, 95, 6480\u20136485.","DOI":"10.1073\/pnas.95.11.6480"},{"key":"key\n\t\t\t\t20171012080755_DDI483C55","doi-asserted-by":"crossref","unstructured":"Zuccato, C., Tartari, M., Crotti, A., Goffredo, D., Valenza, M., Conti, L., Cataudella, T., Leavitt, B.R., Hayden, M.R., Timmusk, T. et\u00a0al. (2003) Huntingtin interacts with REST\/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat. Genet., 35, 76\u201383.","DOI":"10.1038\/ng1219"},{"key":"key\n\t\t\t\t20171012080755_DDI483C56","doi-asserted-by":"crossref","unstructured":"Lorincz, M.T., Detloff, P.J., Albin, R.L. and O'Shea, K.S. (2004) Embryonic stem cells expressing expanded CAG repeats undergo aberrant neuronal differentiation and have persistent Oct-4 and REST\/NRSF expression. Mol. Cell. Neurosci., 26, 135\u2013143.","DOI":"10.1016\/j.mcn.2004.01.016"},{"key":"key\n\t\t\t\t20171012080755_DDI483C57","doi-asserted-by":"crossref","unstructured":"Ballas, N., Battaglioli, E., Atouf, F., Andres, M.E., Chenoweth, J., Anderson, M.E., Burger, C., Moniwa, M., Davie, J.R., Bowers, W.J. et\u00a0al. (2001) Regulation of neuronal traits by a novel transcriptional complex. Neuron, 31, 353\u2013365.","DOI":"10.1016\/S0896-6273(01)00371-3"},{"key":"key\n\t\t\t\t20171012080755_DDI483C58","doi-asserted-by":"crossref","unstructured":"Graveland, G.A., Williams, R.S. and DiFiglia, M. (1985) Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease. Science, 227, 770\u2013773.","DOI":"10.1126\/science.3155875"},{"key":"key\n\t\t\t\t20171012080755_DDI483C59","doi-asserted-by":"crossref","unstructured":"Li, S.H., Cheng, A.L., Li, H. and Li, X.J. (1999) Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin. J. Neurosci., 19, 5159\u20135172.","DOI":"10.1523\/JNEUROSCI.19-13-05159.1999"},{"key":"key\n\t\t\t\t20171012080755_DDI483C60","doi-asserted-by":"crossref","unstructured":"Helmlinger, D., Hardy, S., Abou-Sleymane, G., Eberlin, A., Bowman, A.B., Gahsm\u00fcller, A., Picaud, S., Zoghbi, H.Y., Trottier, Y., Tora, L. and Devys, D. (2006) Glutamine-expanded ataxin-7 alters TFTC\/STAGA recruitment and chromatic structure leading to photoreceptor dysfunction. 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