{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,4]],"date-time":"2025-10-04T22:19:03Z","timestamp":1759616343812},"reference-count":49,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2014,11,17]],"date-time":"2014-11-17T00:00:00Z","timestamp":1416182400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"STEM-HD","award":["FP6, EU; LSHB-CT-2006-037349"],"award-info":[{"award-number":["FP6, EU; LSHB-CT-2006-037349"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology Appl Neurobio"],"published-print":{"date-parts":[[2014,12]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>The repressor element\u20101 silencing transcription factor\/neurone\u2010restrictive silencer factor (<jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">NRSF<\/jats:styled-content>) is a master regulator of neuronal gene expression. <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">NRSF<\/jats:styled-content> functions by recruiting other cofactors to genomic loci that contain the repressor element 1\/neurone restrictive silencer element (<jats:styled-content style=\"fixed-case\">RE<\/jats:styled-content>1\/<jats:styled-content style=\"fixed-case\">NRSE<\/jats:styled-content>) binding motif. In brain, demonstration of <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> protein presence in neurones has remained controversial. However, <jats:styled-content style=\"fixed-case\">RE<\/jats:styled-content>1\/<jats:styled-content style=\"fixed-case\">NRSE<\/jats:styled-content> containing neuronal genes are actively modulated and <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> dysregulation is implicated in Huntington's disease (<jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>). We aimed to investigate <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> distribution in autopsy brain from control and <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Brain tissues from six controls and six <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> cases (<jats:styled-content style=\"fixed-case\">V<\/jats:styled-content>onsattel grade 3 and 4) were investigated using immunohistochemical analysis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> was present in neurones and glial cells of the cortex, caudate nucleus, hippocampus and cerebellum. <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> labelling was mainly cytoplasmic in neurones while preferential nuclear staining of <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> was found in glial cells. We also found that <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> and huntingtin (<jats:styled-content style=\"fixed-case\">HTT<\/jats:styled-content>) colocalize in human neurones. Low levels of cytoplasmic <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> were detected in neurones of the <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> cortex and caudate but no direct relationship between decreased neuronal <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> expression and disease grade was observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These data support the notion of <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content> presence in human brain neurones and glial cells and indicate the importance of developing compounds able to restore <jats:styled-content style=\"fixed-case\">REST<\/jats:styled-content>\u2010regulated transcription of neuronal genes in <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nan.12137","type":"journal-article","created":{"date-parts":[[2014,3,17]],"date-time":"2014-03-17T08:32:27Z","timestamp":1395045147000},"page":"899-910","source":"Crossref","is-referenced-by-count":17,"title":["Repressor element\u20101 silencing transcription factor (<scp>REST<\/scp>) is present in human control and <scp>H<\/scp>untington's disease neurones"],"prefix":"10.1111","volume":"40","author":[{"given":"Davide","family":"Schiffer","sequence":"first","affiliation":[{"name":"Neuro\u2010Bio\u2010Oncology Research Center Policlinico di Monza Foundation  Vercelli"},{"name":"Consorzio per le Neuroscienze University of Pavia  Pavia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valentina","family":"Caldera","sequence":"additional","affiliation":[{"name":"Neuro\u2010Bio\u2010Oncology Research Center Policlinico di Monza Foundation  Vercelli"},{"name":"Consorzio per le Neuroscienze 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