{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T10:24:56Z","timestamp":1768559096783,"version":"3.49.0"},"reference-count":73,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2016,6,19]],"date-time":"2016-06-19T00:00:00Z","timestamp":1466294400000},"content-version":"am","delay-in-days":292,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100000005","name":"U.S. Department of Defense","doi-asserted-by":"publisher","award":["GRANT11188144"],"award-info":[{"award-number":["GRANT11188144"]}],"id":[{"id":"10.13039\/100000005","id-type":"DOI","asserted-by":"publisher"}]},{"name":"ALS Association","award":["GRANT2009"],"award-info":[{"award-number":["GRANT2009"]}]},{"name":"NINDS\/NIH","award":["5R01 MH084880-05"],"award-info":[{"award-number":["5R01 MH084880-05"]}]},{"name":"NINDS\/NIH","award":["R01 NS080882"],"award-info":[{"award-number":["R01 NS080882"]}]},{"name":"NINDS\/NIH","award":["P50 NS071674"],"award-info":[{"award-number":["P50 NS071674"]}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Experimental Neurology"],"published-print":{"date-parts":[[2015,9]]},"DOI":"10.1016\/j.expneurol.2015.06.017","type":"journal-article","created":{"date-parts":[[2015,6,20]],"date-time":"2015-06-20T04:24:43Z","timestamp":1434774283000},"page":"241-250","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":30,"special_numbering":"C","title":["Bromodomain inhibitors regulate the C9ORF72 locus in ALS"],"prefix":"10.1016","volume":"271","author":[{"given":"Zane","family":"Zeier","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rustam","family":"Esanov","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kinsley C.","family":"Belle","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claude-Henry","family":"Volmar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea L.","family":"Johnstone","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Halley","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brooke A.","family":"DeRosa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nathalie","family":"Khoury","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marka","family":"van Blitterswijk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosa","family":"Rademakers","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey","family":"Albert","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shaun P.","family":"Brothers","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joanne","family":"Wuu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Derek M.","family":"Dykxhoorn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Benatar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claes","family":"Wahlestedt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"issue":"3","key":"10.1016\/j.expneurol.2015.06.017_bb0230","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1007\/s00401-013-1149-y","article-title":"Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons","volume":"126","author":"Almeida","year":"2013","journal-title":"Acta Neuropathol."},{"issue":"3","key":"10.1016\/j.expneurol.2015.06.017_bb0310","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1016\/j.cell.2013.07.013","article-title":"BET bromodomains mediate transcriptional pause release in heart failure","volume":"154","author":"Anand","year":"2013","journal-title":"Cell"},{"issue":"5","key":"10.1016\/j.expneurol.2015.06.017_bb0115","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1038\/nrd3674","article-title":"Epigenetic protein families: a new frontier for drug discovery","volume":"11","author":"Arrowsmith","year":"2012","journal-title":"Nat. Rev. Drug Discov."},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0250","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1016\/j.neuron.2013.02.004","article-title":"Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD\/ALS","volume":"77","author":"Ash","year":"2013","journal-title":"Neuron"},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0035","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1016\/0092-8674(91)90514-Y","article-title":"Physical mapping across the Fragile X: hypermethylation and clinical expression of the fragile X syndrome","volume":"64","author":"Bell","year":"1991","journal-title":"Cell"},{"issue":"6","key":"10.1016\/j.expneurol.2015.06.017_bb0020","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1007\/s00401-013-1199-1","article-title":"Reduced C9orf72 gene expression in c9FTD\/ALS is caused by histone trimethylation, an epigenetic event detectable in blood","volume":"126","author":"Belzil","year":"2013","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.expneurol.2015.06.017_bb0165","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.brainres.2014.02.015","article-title":"Characterization of DNA hypermethylation in the cerebellum of c9FTD\/ALS patients","volume":"1584","author":"Belzil","year":"2014","journal-title":"Brain Res."},{"issue":"3","key":"10.1016\/j.expneurol.2015.06.017_bb0090","doi-asserted-by":"crossref","first-page":"e1000017","DOI":"10.1371\/journal.pgen.1000017","article-title":"SIRT1 inhibition alleviates gene silencing in Fragile X mental retardation syndrome","volume":"4","author":"Biacsi","year":"2008","journal-title":"PLoS Genet."},{"issue":"1","key":"10.1016\/j.expneurol.2015.06.017_bb0200","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/0014-4827(71)90599-4","article-title":"An evaluation of the double thymidine block for synchronizing mammalian cells at the G1\u2013S border","volume":"68","author":"Bostock","year":"1971","journal-title":"Exp. Cell Res."},{"issue":"1","key":"10.1016\/j.expneurol.2015.06.017_bb0095","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1093\/hmg\/7.1.109","article-title":"In vitro reactivation of the FMR1 gene involved in fragile X syndrome","volume":"7","author":"Chiurazzi","year":"1998","journal-title":"Hum. Mol. Genet."},{"issue":"35","key":"10.1016\/j.expneurol.2015.06.017_bb0185","doi-asserted-by":"crossref","first-page":"5483","DOI":"10.1038\/sj.onc.1205699","article-title":"5-Azacytidine and 5-aza-2\u2032-deoxycytidine as inhibitors of DNA methylation: mechanistic studies and their implications for cancer therapy","volume":"21","author":"Christman","year":"2002","journal-title":"Oncogene"},{"issue":"2","key":"10.1016\/j.expneurol.2015.06.017_bb0235","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1002\/ana.23946","article-title":"Loss of function of C9orf72 causes motor deficits in a zebrafish model of amyotrophic lateral sclerosis","volume":"74","author":"Ciura","year":"2013","journal-title":"Ann. Neurol."},{"issue":"7370","key":"10.1016\/j.expneurol.2015.06.017_bb0140","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1038\/nature10509","article-title":"Inhibition of BET recruitment to chromatin as an effective treatment for MLL-fusion leukaemia","volume":"478","author":"Dawson","year":"2011","journal-title":"Nature"},{"key":"10.1016\/j.expneurol.2015.06.017_bb0335","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1038\/nature10509","article-title":"Inhibition of BET recruitment to chromatin as an effective treatment for MLL-fusion leukaemia","volume":"478","author":"Dawson","year":"2011","journal-title":"Nature (London)"},{"issue":"2","key":"10.1016\/j.expneurol.2015.06.017_bb0005","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.neuron.2011.09.011","article-title":"Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS","volume":"72","author":"DeJesus-Hernandez","year":"2011","journal-title":"Neuron"},{"issue":"6","key":"10.1016\/j.expneurol.2015.06.017_bb0145","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1016\/j.cell.2011.08.017","article-title":"BET bromodomain inhibition as a therapeutic strategy to target c-Myc","volume":"146","author":"Delmore","year":"2011","journal-title":"Cell"},{"issue":"1","key":"10.1016\/j.expneurol.2015.06.017_bb0110","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1002\/acn3.147","article-title":"The promise and perils of HDAC inhibitors in neurodegeneration","volume":"2","author":"Didonna","year":"2015","journal-title":"Ann. Clin. Transl. Neurol."},{"issue":"2","key":"10.1016\/j.expneurol.2015.06.017_bb0060","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1016\/j.neuron.2013.10.015","article-title":"RNA toxicity from the ALS\/FTD C9ORF72 expansion is mitigated by antisense intervention","volume":"80","author":"Donnelly","year":"2013","journal-title":"Neuron"},{"issue":"3","key":"10.1016\/j.expneurol.2015.06.017_bb0360","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1016\/j.scr.2013.01.009","article-title":"EZ spheres: a stable and expandable culture system for the generation of pre-rosette multipotent stem cells from human ESCs and iPSCs","volume":"10","author":"Ebert","year":"2013","journal-title":"Stem Cell Res."},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0050","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1002\/ana.22671","article-title":"FXN methylation predicts expression and clinical outcome in Friedreich ataxia","volume":"71","author":"Evans-Galea","year":"2012","journal-title":"Ann. Neurol."},{"issue":"7327","key":"10.1016\/j.expneurol.2015.06.017_bb0125","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1038\/nature09504","article-title":"Selective inhibition of BET bromodomains","volume":"468","author":"Filippakopoulos","year":"2010","journal-title":"Nature"},{"issue":"6","key":"10.1016\/j.expneurol.2015.06.017_bb0340","doi-asserted-by":"crossref","first-page":"1878","DOI":"10.1016\/j.bmc.2011.10.080","article-title":"Benzodiazepines and benzotriazepines as protein interaction inhibitors targeting bromodomains of the BET family","volume":"20","author":"Filippakopoulos","year":"2012","journal-title":"Bioorg. Med. Chem."},{"issue":"7453","key":"10.1016\/j.expneurol.2015.06.017_bb0315","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1038\/nature12147","article-title":"The bromodomain protein Brd4 insulates chromatin from DNA damage signalling","volume":"498","author":"Floyd","year":"2013","journal-title":"Nature"},{"issue":"3","key":"10.1016\/j.expneurol.2015.06.017_bb0255","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1007\/s00401-013-1147-0","article-title":"Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia","volume":"126","author":"Fratta","year":"2013","journal-title":"Acta Neuropathol."},{"issue":"1","key":"10.1016\/j.expneurol.2015.06.017_bb0240","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1016\/S1474-4422(11)70261-7","article-title":"A C9orf72 promoter repeat expansion in a Flanders\u2013Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study","volume":"11","author":"Gijselinck","year":"2012","journal-title":"Lancet Neurol."},{"issue":"5","key":"10.1016\/j.expneurol.2015.06.017_bb0030","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1055\/s-0031-1299786","article-title":"Epigenetics in nucleotide repeat expansion disorders","volume":"31","author":"He","year":"2011","journal-title":"Semin. Neurol."},{"issue":"5","key":"10.1016\/j.expneurol.2015.06.017_bb0260","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1128\/MCB.01365-08","article-title":"Brd4 coactivates transcriptional activation of NF-kappaB via specific binding to acetylated RelA","volume":"29","author":"Huang","year":"2009","journal-title":"Mol. Cell. Biol."},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0285","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1016\/j.molcel.2005.06.027","article-title":"The bromodomain protein Brd4 is a positive regulatory component of P-TEFb and stimulates RNA polymerase II-dependent transcription","volume":"19","author":"Jang","year":"2005","journal-title":"Mol. Cell"},{"issue":"2","key":"10.1016\/j.expneurol.2015.06.017_bb0350","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/j.mcn.2012.04.008","article-title":"A chemical genetic approach identifies piperazine antipsychotics as promoters of CNS neurite growth on inhibitory substrates","volume":"50","author":"Johnstone","year":"2012","journal-title":"Mol. Cell. Neurosci."},{"issue":"10","key":"10.1016\/j.expneurol.2015.06.017_bb0345","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1038\/nbt.1678","article-title":"Epigenetic modifications as therapeutic targets","volume":"28","author":"Kelly","year":"2010","journal-title":"Nat. Biotechnol."},{"issue":"1","key":"10.1016\/j.expneurol.2015.06.017_bb0055","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1186\/1868-7083-4-2","article-title":"Is Friedreich ataxia an epigenetic disorder?","volume":"4","author":"Kumari","year":"2012","journal-title":"Clin. Epigenet."},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0225","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1093\/bioinformatics\/bts725","article-title":"The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs","volume":"29","author":"Levine","year":"2013","journal-title":"Bioinformatics"},{"issue":"2","key":"10.1016\/j.expneurol.2015.06.017_bb0365","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1038\/nbt1063","article-title":"Specification of motoneurons from human embryonic stem cells","volume":"23","author":"Li","year":"2005","journal-title":"Nat. Biotechnol."},{"issue":"7","key":"10.1016\/j.expneurol.2015.06.017_bb0280","doi-asserted-by":"crossref","first-page":"1581","DOI":"10.1016\/j.cell.2013.10.056","article-title":"Brd4 and JMJD6-associated anti-pause enhancers in regulation of transcriptional pause release","volume":"155","author":"Liu","year":"2013","journal-title":"Cell"},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0170","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1007\/s00401-014-1286-y","article-title":"C9orf72 hypermethylation protects against repeat expansion-associated pathology in ALS\/FTD","volume":"128","author":"Liu","year":"2014","journal-title":"Acta Neuropathol."},{"issue":"2","key":"10.1016\/j.expneurol.2015.06.017_bb0300","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1016\/j.cell.2013.03.036","article-title":"Selective inhibition of tumor oncogenes by disruption of super-enhancers","volume":"153","author":"Loven","year":"2013","journal-title":"Cell"},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0015","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/S1474-4422(12)70043-1","article-title":"Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study","volume":"11","author":"Majounie","year":"2012","journal-title":"Lancet Neurol."},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0330","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1016\/j.cell.2012.06.045","article-title":"Small-molecule inhibition of BRDT for male contraception","volume":"150","author":"Matzuk","year":"2012","journal-title":"Cell"},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0355","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1016\/0896-6273(95)90172-8","article-title":"Characterization of the signaling interactions that promote the survival and growth of developing retinal ganglion cells in culture","volume":"15","author":"Meyer-Franke","year":"1995","journal-title":"Neuron"},{"issue":"19","key":"10.1016\/j.expneurol.2015.06.017_bb0325","doi-asserted-by":"crossref","first-page":"7501","DOI":"10.1021\/jm401088k","article-title":"Discovery of epigenetic regulator I-BET762: lead optimization to afford a clinical candidate inhibitor of the BET bromodomains","volume":"56","author":"Mirguet","year":"2013","journal-title":"J. Med. Chem."},{"issue":"14","key":"10.1016\/j.expneurol.2015.06.017_bb0190","doi-asserted-by":"crossref","first-page":"9040","DOI":"10.1074\/jbc.M707603200","article-title":"The bromodomain protein Brd4 stimulates G1 gene transcription and promotes progression to S phase","volume":"283","author":"Mochizuki","year":"2008","journal-title":"J. Biol. Chem."},{"issue":"6125","key":"10.1016\/j.expneurol.2015.06.017_bb0245","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1126\/science.1232927","article-title":"The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD\/ALS","volume":"339","author":"Mori","year":"2013","journal-title":"Science"},{"issue":"7327","key":"10.1016\/j.expneurol.2015.06.017_bb0130","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1038\/nature09589","article-title":"Suppression of inflammation by a synthetic histone mimic","volume":"468","author":"Nicodeme","year":"2010","journal-title":"Nature"},{"issue":"5","key":"10.1016\/j.expneurol.2015.06.017_bb0155","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/ahg.12033","article-title":"C9ORF72 Intermediate repeat copies are a significant risk factor for Parkinson disease","volume":"77","author":"Nuytemans","year":"2013","journal-title":"Ann. Hum. Genet."},{"issue":"14","key":"10.1016\/j.expneurol.2015.06.017_bb0150","doi-asserted-by":"crossref","first-page":"2843","DOI":"10.1182\/blood-2012-02-413021","article-title":"BET bromodomain inhibition targets both c-Myc and IL7R in high-risk acute lymphoblastic leukemia","volume":"120","author":"Ott","year":"2012","journal-title":"Blood"},{"issue":"12","key":"10.1016\/j.expneurol.2015.06.017_bb0265","doi-asserted-by":"crossref","first-page":"2497","DOI":"10.1128\/MCB.01180-12","article-title":"BRD4 coordinates recruitment of pause release factor P-TEFb and the pausing complex NELF\/DSIF to regulate transcription elongation of interferon-stimulated genes","volume":"33","author":"Patel","year":"2013","journal-title":"Mol. Cell. Biol."},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0205","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1016\/0092-8674(91)90125-I","article-title":"Absence of expression of the FMR-1 gene in fragile X syndrome","volume":"66","author":"Pieretti","year":"1991","journal-title":"Cell"},{"issue":"14","key":"10.1016\/j.expneurol.2015.06.017_bb0100","doi-asserted-by":"crossref","first-page":"3278","DOI":"10.1093\/nar\/gkf434","article-title":"Quantitative analysis of DNA demethylation and transcriptional reactivation of the FMR1 gene in fragile X cells treated with 5-azadeoxycytidine","volume":"30","author":"Pietrobono","year":"2002","journal-title":"Nucleic Acids Res."},{"issue":"3","key":"10.1016\/j.expneurol.2015.06.017_bb0120","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.tips.2011.12.002","article-title":"Place your BETs: the therapeutic potential of bromodomains","volume":"33","author":"Prinjha","year":"2012","journal-title":"Trends Pharmacol. Sci."},{"issue":"13","key":"10.1016\/j.expneurol.2015.06.017_bb0275","doi-asserted-by":"crossref","first-page":"2641","DOI":"10.1128\/MCB.01341-10","article-title":"The Brd4 extraterminal domain confers transcription activation independent of pTEFb by recruiting multiple proteins, including NSD3","volume":"31","author":"Rahman","year":"2011","journal-title":"Mol. Cell. Biol."},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0075","doi-asserted-by":"crossref","first-page":"e1958","DOI":"10.1371\/journal.pone.0001958","article-title":"HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model","volume":"3","author":"Rai","year":"2008","journal-title":"PLoS One"},{"issue":"1","key":"10.1016\/j.expneurol.2015.06.017_bb0070","doi-asserted-by":"crossref","first-page":"e8825","DOI":"10.1371\/journal.pone.0008825","article-title":"Two new pimelic diphenylamide HDAC inhibitors induce sustained frataxin upregulation in cells from Friedreich's ataxia patients and in a mouse model","volume":"5","author":"Rai","year":"2010","journal-title":"PLoS One"},{"issue":"2","key":"10.1016\/j.expneurol.2015.06.017_bb0010","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/j.neuron.2011.09.010","article-title":"A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD","volume":"72","author":"Renton","year":"2011","journal-title":"Neuron"},{"issue":"1","key":"10.1016\/j.expneurol.2015.06.017_bb0175","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1007\/s00401-014-1365-0","article-title":"Hypermethylation of repeat expanded C9orf72 is a clinical and molecular disease modifier","volume":"129","author":"Russ","year":"2015","journal-title":"Acta Neuropathol."},{"issue":"3","key":"10.1016\/j.expneurol.2015.06.017_bb0105","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1016\/j.nbd.2011.02.016","article-title":"Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model","volume":"42","author":"Sandi","year":"2011","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.expneurol.2015.06.017_bb0080","doi-asserted-by":"crossref","first-page":"165","DOI":"10.3389\/fgene.2014.00165","article-title":"Epigenetic-based therapies for Friedreich ataxia","volume":"5","author":"Sandi","year":"2014","journal-title":"Front. Genet."},{"issue":"5","key":"10.1016\/j.expneurol.2015.06.017_bb0270","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1016\/j.molcel.2014.05.016","article-title":"The mechanisms behind the therapeutic activity of BET bromodomain inhibition","volume":"54","author":"Shi","year":"2014","journal-title":"Mol. Cell"},{"issue":"8","key":"10.1016\/j.expneurol.2015.06.017_bb0215","doi-asserted-by":"crossref","first-page":"e23648","DOI":"10.1371\/journal.pone.0023648","article-title":"Testing the FMR1 promoter for mosaicism in DNA methylation among CpG sites, strands, and cells in FMR1-expressing males with fragile X syndrome","volume":"6","author":"Stoger","year":"2011","journal-title":"PLoS One"},{"issue":"7","key":"10.1016\/j.expneurol.2015.06.017_bb0210","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1136\/jmg.38.7.453","article-title":"A majority of Fragile X males with methylated, full mutation alleles have significant levels of FMR1 messenger RNA","volume":"38","author":"Tassone","year":"2001","journal-title":"J. Med. Genet."},{"issue":"12","key":"10.1016\/j.expneurol.2015.06.017_bb0045","doi-asserted-by":"crossref","first-page":"e1001240","DOI":"10.1371\/journal.pgen.1001240","article-title":"Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of Fragile X tremor ataxia syndrome","volume":"6","author":"Todd","year":"2010","journal-title":"PLoS Genet."},{"issue":"2","key":"10.1016\/j.expneurol.2015.06.017_bb0320","doi-asserted-by":"crossref","first-page":"142","DOI":"10.3109\/10409238.2014.999192","article-title":"Repeat instability during DNA repair: insights from model systems","volume":"50","author":"Usdin","year":"2015","journal-title":"Crit. Rev. Biochem. Mol. Biol."},{"issue":"10","key":"10.1016\/j.expneurol.2015.06.017_bb0160","doi-asserted-by":"crossref","first-page":"978","DOI":"10.1016\/S1474-4422(13)70210-2","article-title":"Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort study","volume":"12","author":"van Blitterswijk","year":"2013","journal-title":"Lancet Neurol."},{"issue":"6310","key":"10.1016\/j.expneurol.2015.06.017_bb0040","doi-asserted-by":"crossref","first-page":"624","DOI":"10.1038\/349624a0","article-title":"Abnormal pattern detected in Fragile-X patients by pulsed-field gel electrophoresis","volume":"349","author":"Vincent","year":"1991","journal-title":"Nature"},{"issue":"1","key":"10.1016\/j.expneurol.2015.06.017_bb0085","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1016\/j.nepig.2014.10.002","article-title":"Histone deacetylases (HDACs) and brain function","volume":"1","author":"Volmar","year":"2015","journal-title":"Neuroepigenetics"},{"issue":"5","key":"10.1016\/j.expneurol.2015.06.017_bb0295","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1016\/j.molcel.2012.12.006","article-title":"Phospho switch triggers Brd4 chromatin binding and activator recruitment for gene-specific targeting","volume":"49","author":"Wu","year":"2013","journal-title":"Mol. Cell"},{"issue":"6","key":"10.1016\/j.expneurol.2015.06.017_bb0025","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1016\/j.ajhg.2013.04.017","article-title":"Hypermethylation of the CpG island near the G4C2 repeat in ALS with a C9orf72 expansion","volume":"92","author":"Xi","year":"2013","journal-title":"Am. J. Hum. Genet."},{"issue":"21","key":"10.1016\/j.expneurol.2015.06.017_bb0180","doi-asserted-by":"crossref","first-page":"5630","DOI":"10.1093\/hmg\/ddu279","article-title":"Hypermethylation of the CpG-island near the C9orf72 G(4)C(2)-repeat expansion in FTLD patients","volume":"23","author":"Xi","year":"2014","journal-title":"Hum. Mol. Genet."},{"issue":"5","key":"10.1016\/j.expneurol.2015.06.017_bb0065","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1007\/s00401-015-1401-8","article-title":"The C9orf72 repeat expansion itself is methylated in ALS and FTLD patients","volume":"129","author":"Xi","year":"2015","journal-title":"Acta Neuropathol."},{"issue":"4","key":"10.1016\/j.expneurol.2015.06.017_bb0290","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1016\/j.molcel.2005.06.029","article-title":"Recruitment of P-TEFb for stimulation of transcriptional elongation by the bromodomain protein Brd4","volume":"19","author":"Yang","year":"2005","journal-title":"Mol. Cell"},{"issue":"3","key":"10.1016\/j.expneurol.2015.06.017_bb0195","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1128\/MCB.01020-07","article-title":"Brd4 recruits P-TEFb to chromosomes at late mitosis to promote G1 gene expression and cell cycle progression","volume":"28","author":"Yang","year":"2008","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.expneurol.2015.06.017_bb0220","doi-asserted-by":"crossref","first-page":"283","DOI":"10.3389\/fgene.2012.00283","article-title":"Discovery of Novel DENN proteins: implications for the evolution of eukaryotic intracellular membrane structures and human disease","volume":"3","author":"Zhang","year":"2012","journal-title":"Front. Genet."},{"issue":"51","key":"10.1016\/j.expneurol.2015.06.017_bb0305","doi-asserted-by":"crossref","first-page":"43137","DOI":"10.1074\/jbc.M112.413047","article-title":"Bromodomain-containing protein 4 (BRD4) regulates RNA polymerase II serine 2 phosphorylation in human CD4+ T cells","volume":"287","author":"Zhang","year":"2012","journal-title":"J. Biol. Chem."},{"issue":"7370","key":"10.1016\/j.expneurol.2015.06.017_bb0135","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1038\/nature10334","article-title":"RNAi screen identifies Brd4 as a therapeutic target in acute myeloid leukaemia","volume":"478","author":"Zuber","year":"2011","journal-title":"Nature"}],"container-title":["Experimental Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488615300273?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488615300273?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,6,9]],"date-time":"2024-06-09T16:39:12Z","timestamp":1717951152000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0014488615300273"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,9]]},"references-count":73,"alternative-id":["S0014488615300273"],"URL":"https:\/\/doi.org\/10.1016\/j.expneurol.2015.06.017","relation":{},"ISSN":["0014-4886"],"issn-type":[{"value":"0014-4886","type":"print"}],"subject":[],"published":{"date-parts":[[2015,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Bromodomain inhibitors regulate the C9ORF72 locus in ALS","name":"articletitle","label":"Article Title"},{"value":"Experimental Neurology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.expneurol.2015.06.017","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2015 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}