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Del Toro","year":"2009","journal-title":"Mol Biol Cell"},{"key":"ref78","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.bbadis.2012.09.001","article-title":"Mutant huntingtin regulates EGF receptor fate in non-neuronal cells lacking wild-type protein","volume":"1832","author":"M a B Melone","year":"2013","journal-title":"Biochim Biophys Acta"},{"key":"ref79","doi-asserted-by":"crossref","first-page":"6703","DOI":"10.1074\/jbc.M110338200","article-title":"Expression of full-length polyglutamine-expanded Huntingtin disrupts growth factor receptor signaling in rat pheochromocytoma (PC12) cells","volume":"277","author":"C Song","year":"2002","journal-title":"J Biol Chem"},{"key":"ref80","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1093\/hmg\/ddi067","article-title":"Expanded polyglutamine peptides disrupt EGF receptor signaling and glutamate transporter expression in Drosophila","volume":"14","author":"J-C Li\u00e9vens","year":"2005","journal-title":"Hum Mol 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