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Repressor Element\u20101 Silencing Transcription Factor (<jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content>) is a repressor whose association with Htt in the cytoplasm is disrupted in <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>, leading to increased nuclear <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> and concomitant repression of several neuronal\u2010specific genes, including brain\u2010derived neurotrophic factor (<jats:italic>Bdnf<\/jats:italic>). Here, we explored a wide set of <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> dysregulated genes to identify direct <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> targets whose expression is altered in a cellular model of <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> but that can be rescued by knock\u2010down of <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> activity. We found many direct <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> target genes encoding proteins important for nervous system development, including a cohort involved in synaptic transmission, at least two of which can be rescued at the protein level by <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> knock\u2010down. We also identified several micro<jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content>s (mi<jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content>s) whose aberrant repression is directly mediated by <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content>, including miR\u2010137, which has not previously been shown to be a direct <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> target in mouse. These data provide evidence of the contribution of inappropriate <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content>\u2010mediated transcriptional repression to the widespread changes in coding and non\u2010coding gene expression in a cellular model of <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> that may affect normal neuronal function and survival.<\/jats:p>","DOI":"10.1111\/jnc.12090","type":"journal-article","created":{"date-parts":[[2012,11,12]],"date-time":"2012-11-12T12:02:29Z","timestamp":1352721749000},"page":"418-430","source":"Crossref","is-referenced-by-count":73,"title":["Dysregulation of <scp>REST<\/scp>\u2010regulated coding and non\u2010coding <scp>RNA<\/scp>s in a cellular model of Huntington's disease"],"prefix":"10.1111","volume":"124","author":[{"given":"Chiara","family":"Soldati","sequence":"first","affiliation":[{"name":"Department of Neuroscience Centre for the Cellular Basis of Behaviour Institute of Psychiatry King's College London London UK"},{"name":"Department of Biology and Biotechnology \u201cCharles Darwin\u201d University of Rome \u201cLa Sapienza\u201d Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Bithell","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Centre for the Cellular Basis of Behaviour Institute of Psychiatry King's College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Johnston","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Centre for the Cellular Basis of Behaviour Institute of Psychiatry King's College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kee\u2010Yew","family":"Wong","sequence":"additional","affiliation":[{"name":"Genome Institute of Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lawrence W.","family":"Stanton","sequence":"additional","affiliation":[{"name":"Genome Institute of Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noel J.","family":"Buckley","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Centre for the Cellular Basis of Behaviour Institute of Psychiatry King's College London London 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