{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T06:21:36Z","timestamp":1790749296553,"version":"4.1.0"},"reference-count":48,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2018,9,19]],"date-time":"2018-09-19T00:00:00Z","timestamp":1537315200000},"content-version":"am","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS076631"],"award-info":[{"award-number":["NS076631"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS084475"],"award-info":[{"award-number":["NS084475"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006458","name":"Children's Hospital of Philadelphia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006458","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"DOI":"10.1093\/hmg\/ddy331","type":"journal-article","created":{"date-parts":[[2018,9,17]],"date-time":"2018-09-17T07:14:34Z","timestamp":1537168474000},"source":"Crossref","is-referenced-by-count":19,"title":["The long non-coding RNA NEAT1 is elevated in polyglutamine repeat expansion diseases and protects from disease gene-dependent toxicities"],"prefix":"10.1093","author":[{"given":"Congsheng","family":"Cheng","sequence":"first","affiliation":[{"name":"Raymond G. Perelman Center for Cellular and Molecular Therapeutics, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan M","family":"Spengler","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megan S","family":"Keiser","sequence":"additional","affiliation":[{"name":"Raymond G. Perelman Center for Cellular and Molecular Therapeutics, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alejandro Mas","family":"Monteys","sequence":"additional","affiliation":[{"name":"Raymond G. Perelman Center for Cellular and Molecular Therapeutics, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julianne M","family":"Rieders","sequence":"additional","affiliation":[{"name":"Raymond G. Perelman Center for Cellular and Molecular Therapeutics, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shyam","family":"Ramachandran","sequence":"additional","affiliation":[{"name":"Raymond G. Perelman Center for Cellular and Molecular Therapeutics, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beverly L","family":"Davidson","sequence":"additional","affiliation":[{"name":"Raymond G. Perelman Center for Cellular and Molecular Therapeutics, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, USA"},{"name":"Department of Pathology and Laboratory Medicine, Children\u2019s Hospital of Philadelphia and University of Pennsylvania, Philadelphia, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2018,9,19]]},"reference":[{"key":"key\n\t\t\t\t20181010112241222_ref1","doi-asserted-by":"crossref","first-page":"1199","DOI":"10.1021\/acs.biochem.6b00936","article-title":"Proteins containing expanded polyglutamine tracts and neurodegenerative disease","volume":"56","author":"Adegbuyiro","year":"2017","journal-title":"Biochemistry"},{"key":"key\n\t\t\t\t20181010112241222_ref2","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/j.neuron.2014.12.019","article-title":"Reinstating aberrant mTORC1 activity in Huntington\u2019s disease mice improves disease phenotypes","volume":"85","author":"Lee","year":"2015","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20181010112241222_ref3","doi-asserted-by":"crossref","first-page":"3454","DOI":"10.1093\/hmg\/ddw187","article-title":"Transcriptome sequencing reveals aberrant alternative splicing in Huntington's disease","volume":"25","author":"Lin","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20181010112241222_ref4","doi-asserted-by":"crossref","first-page":"e0143563","DOI":"10.1371\/journal.pone.0143563","article-title":"RNA sequence analysis of human Huntington disease brain reveals an extensive increase in inflammatory and developmental gene expression","volume":"10","author":"Labadorf","year":"2015","journal-title":"PLoS One"},{"key":"key\n\t\t\t\t20181010112241222_ref5","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1038\/nn.4532","article-title":"Developmental alterations in Huntington's disease neural cells and pharmacological rescue in cells and mice","volume":"20","author":"iPSC Consortium","year":"2017","journal-title":"Nat. Neurosci."},{"key":"key\n\t\t\t\t20181010112241222_ref6","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1186\/s12864-016-3114-3","article-title":"Novel microRNA discovery using small RNA sequencing in post-mortem human brain","volume":"17","author":"Wake","year":"2016","journal-title":"BMC Genomics"},{"key":"key\n\t\t\t\t20181010112241222_ref7","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1038\/nrm3679","article-title":"RNA in unexpected places: long non-coding RNA functions in diverse cellular contexts","volume":"14","author":"Geisler","year":"2013","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"key\n\t\t\t\t20181010112241222_ref8","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.gene.2014.06.043","article-title":"The ways of action of long non-coding RNAs in cytoplasm and nucleus","volume":"547","author":"Zhang","year":"2014","journal-title":"Gene"},{"key":"key\n\t\t\t\t20181010112241222_ref9","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1038\/nrn.2017.90","article-title":"Noncoding RNAs in neurodegeneration","volume":"18","author":"Salta","year":"2017","journal-title":"Nat. Rev. Neurosci."},{"key":"key\n\t\t\t\t20181010112241222_ref10","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1016\/j.nbd.2015.07.011","article-title":"Long noncoding RNAs in TDP-43 and FUS\/TLS-related frontotemporal lobar degeneration (FTLD)","volume":"82","author":"Lourenco","year":"2015","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20181010112241222_ref11","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1007\/s11515-016-1433-z","article-title":"Cellular, physiological and pathological aspects of the long non-coding RNA NEAT1","volume":"11","author":"Lo","year":"2016","journal-title":"Front Biol (Beijing)"},{"key":"key\n\t\t\t\t20181010112241222_ref12","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.gendis.2017.11.003","article-title":"Pan-cancer analysis of long non-coding RNA NEAT1 in various cancers","volume":"5","author":"Li","year":"2018","journal-title":"Genes Dis."},{"key":"key\n\t\t\t\t20181010112241222_ref13","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.nbd.2011.12.006","article-title":"Long non-coding RNAs in Huntington's disease neurodegeneration","volume":"46","author":"Johnson","year":"2012","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20181010112241222_ref14","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1007\/s12035-016-9928-9","article-title":"Altered expression of the long noncoding RNA NEAT1 in Huntington\u2019s disease","volume":"54","author":"Sunwoo","year":"2016","journal-title":"Mol. Neurobiol."},{"key":"key\n\t\t\t\t20181010112241222_ref15","doi-asserted-by":"crossref","first-page":"13315","DOI":"10.1093\/nar\/gku979","article-title":"Single nucleotide seed modification restores in vivo tolerability of a toxic artificial miRNA sequence in the mouse brain","volume":"42","author":"Monteys","year":"2014","journal-title":"Nucleic Acids Res."},{"key":"key\n\t\t\t\t20181010112241222_ref16","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1083\/jcb.201011110","article-title":"Paraspeckles are subpopulation-specific nuclear bodies that are not essential in mice","volume":"193","author":"Nakagawa","year":"2011","journal-title":"J. Cell Biol."},{"key":"key\n\t\t\t\t20181010112241222_ref17","doi-asserted-by":"crossref","first-page":"3975","DOI":"10.1093\/hmg\/ddw238","article-title":"Pridopidine activates neuroprotective pathways impaired in Huntington disease","volume":"25","author":"Geva","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20181010112241222_ref18","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1038\/nm.4003","article-title":"PPAR-\u03b4 is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically","volume":"22","author":"Dickey","year":"2016","journal-title":"Nat. Med."},{"key":"key\n\t\t\t\t20181010112241222_ref19","doi-asserted-by":"crossref","first-page":"1521","DOI":"10.1111\/j.1471-4159.2005.03515.x","article-title":"3-Nitropropionic acid: a mitochondrial toxin to uncover physiopathological mechanisms underlying striatal degeneration in Huntington's disease","volume":"95","author":"Brouillet","year":"2005","journal-title":"J. Neurochem."},{"key":"key\n\t\t\t\t20181010112241222_ref20","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1038\/nrg.2015.10","article-title":"Unique features of long non-coding RNA biogenesis and function","volume":"17","author":"Quinn","year":"2016","journal-title":"Nat. Rev. Genet."},{"key":"key\n\t\t\t\t20181010112241222_ref21","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.conb.2017.09.004","article-title":"Deciphering MECP2-associated disorders: disrupted circuits and the hope for repair","volume":"48","author":"Qiu","year":"2017","journal-title":"Curr. Opin. Neurobiol."},{"key":"key\n\t\t\t\t20181010112241222_ref22","doi-asserted-by":"crossref","first-page":"1741","DOI":"10.4161\/rna.26921","article-title":"Chromatin context and ncRNA highlight targets of MeCP2 in brain","volume":"10","author":"Maxwell","year":"2013","journal-title":"RNA Biol."},{"key":"key\n\t\t\t\t20181010112241222_ref23","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1016\/j.molcel.2016.06.029","article-title":"Comprehensive identification of RNA-binding domains in human cells","volume":"63","author":"Castello","year":"2016","journal-title":"Mol. Cell"},{"key":"key\n\t\t\t\t20181010112241222_ref24","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1261\/rna.058115.116","article-title":"Quantifying RNA binding sites transcriptome-wide using DO-RIP-seq","volume":"23","author":"Nicholson","year":"2017","journal-title":"RNA"},{"key":"key\n\t\t\t\t20181010112241222_ref25","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1038\/nature19342","article-title":"m(6)A RNA methylation promotes XIST-mediated transcriptional repression","volume":"537","author":"Patil","year":"2016","journal-title":"Nature"},{"key":"key\n\t\t\t\t20181010112241222_ref26","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1038\/nrd.2016.117","article-title":"Non-coding RNAs as drug targets","volume":"16","author":"Matsui","year":"2017","journal-title":"Nat. Rev. Drug Discov."},{"key":"key\n\t\t\t\t20181010112241222_ref27","doi-asserted-by":"crossref","first-page":"e00596","DOI":"10.1128\/mBio.00596-12","article-title":"NEAT1 long noncoding RNA and paraspeckle bodies modulate HIV-1 posttranscriptional expression","volume":"4","author":"Zhang","year":"2013","journal-title":"MBio"},{"key":"key\n\t\t\t\t20181010112241222_ref28","doi-asserted-by":"crossref","first-page":"2785","DOI":"10.1002\/ijc.30689","article-title":"Long non-coding RNA NEAT1 is a transcriptional target of p53 and modulates p53-induced transactivation and tumor-suppressor function","volume":"140","author":"Idogawa","year":"2017","journal-title":"Int. J. Cancer"},{"key":"key\n\t\t\t\t20181010112241222_ref29","doi-asserted-by":"crossref","first-page":"1","DOI":"10.2741\/e715","article-title":"The building process of the functional paraspeckle with long non-coding RNAs","volume":"7","author":"Yamazaki","year":"2015","journal-title":"Front. Biosci."},{"key":"key\n\t\t\t\t20181010112241222_ref30","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1038\/ncb2140","article-title":"Direct visualization of the co-transcriptional assembly of a nuclear body by noncoding RNAs","volume":"13","author":"Mao","year":"2011","journal-title":"Nat. Cell Biol."},{"key":"key\n\t\t\t\t20181010112241222_ref31","first-page":"73","article-title":"Long noncoding RNAs: critical regulators for cell lineage commitment in the central nervous system","author":"Dong","year":"2016"},{"key":"key\n\t\t\t\t20181010112241222_ref32","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1126\/science.aar7432","article-title":"mRNA structure determines specificity of a polyQ-driven phase separation","volume":"360","author":"Langdon","year":"2018","journal-title":"Science"},{"key":"key\n\t\t\t\t20181010112241222_ref33","doi-asserted-by":"crossref","first-page":"1447","DOI":"10.15252\/embj.201695848","article-title":"Unusual semi-extractability as a hallmark of nuclear body-associated architectural noncoding RNAs","volume":"36","author":"Chujo","year":"2017","journal-title":"EMBO J."},{"key":"key\n\t\t\t\t20181010112241222_ref34","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1038\/nsmb.3455","article-title":"NEAT1 scaffolds RNA-binding proteins and the microprocessor to globally enhance pri-miRNA processing","volume":"24","author":"Jiang","year":"2017","journal-title":"Nat. Struct. Mol. Biol."},{"key":"key\n\t\t\t\t20181010112241222_ref35","doi-asserted-by":"crossref","first-page":"14341","DOI":"10.1523\/JNEUROSCI.2390-08.2008","article-title":"The bifunctional microRNA miR-9\/miR-9* regulates REST and CoREST and is downregulated in Huntington's disease","volume":"28","author":"Packer","year":"2008","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20181010112241222_ref36","doi-asserted-by":"crossref","first-page":"2225","DOI":"10.1093\/hmg\/ddr111","article-title":"Hsa-miR-34b is a plasma-stable microRNA that is elevated in pre-manifest Huntington's disease","volume":"20","author":"Gaughwin","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20181010112241222_ref37","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/j.nbd.2013.01.019","article-title":"Altered Purkinje cell miRNA expression and SCA1 pathogenesis","volume":"54","author":"Rodriguez-Lebron","year":"2013","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20181010112241222_ref38","doi-asserted-by":"crossref","first-page":"1038","DOI":"10.1016\/j.molcel.2018.05.019","article-title":"Functional domains of NEAT1 architectural lncRNA induce paraspeckle assembly through phase separation","volume":"70","author":"Yamazaki","year":"2018","journal-title":"Mol. Cell"},{"key":"key\n\t\t\t\t20181010112241222_ref39","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1038\/nature22386","article-title":"RNA phase transitions in repeat expansion disorders","volume":"546","author":"Jain","year":"2017","journal-title":"Nature"},{"key":"key\n\t\t\t\t20181010112241222_ref40","doi-asserted-by":"crossref","first-page":"3555","DOI":"10.1093\/brain\/awv292","article-title":"Broad distribution of ataxin 1 silencing in rhesus cerebella for spinocerebellar ataxia type 1 therapy","volume":"138","author":"Keiser","year":"2015","journal-title":"Brain"},{"key":"key\n\t\t\t\t20181010112241222_ref41","doi-asserted-by":"crossref","first-page":"2344","DOI":"10.1093\/hmg\/ddr127","article-title":"Striatal neurons expressing full-length mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis","volume":"20","author":"Reis","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20181010112241222_ref42","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1038\/nm.4135","article-title":"p53 induces formation of NEAT1 lncRNA-containing paraspeckles that modulate replication stress response and chemosensitivity","volume":"22","author":"Adriaens","year":"2016","journal-title":"Nat. Med."},{"key":"key\n\t\t\t\t20181010112241222_ref43","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1038\/nature25757","article-title":"Sequences enriched in Alu repeats drive nuclear localization of long RNAs in human cells","volume":"555","author":"Lubelsky","year":"2018","journal-title":"Nature"},{"key":"key\n\t\t\t\t20181010112241222_ref44","first-page":"7120","article-title":"Elucidation of transcriptome-wide microRNA binding sites in human cardiac tissues by Ago2 HITS-CLIP","volume":"44","author":"Spengler","year":"2016","journal-title":"Nucleic Acids Res."},{"key":"key\n\t\t\t\t20181010112241222_ref45","doi-asserted-by":"crossref","first-page":"7576","DOI":"10.1038\/ncomms8576","article-title":"A post-transcriptional mechanism pacing expression of neural genes with precursor cell differentiation status","volume":"6","author":"Dai","year":"2015","journal-title":"Nat. Commun."},{"key":"key\n\t\t\t\t20181010112241222_ref46","doi-asserted-by":"crossref","first-page":"792","DOI":"10.1016\/j.molcel.2013.08.017","article-title":"Circular intronic long noncoding RNAs","volume":"51","author":"Zhang","year":"2013","journal-title":"Mol. Cell"},{"key":"key\n\t\t\t\t20181010112241222_ref47","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1111\/j.1476-5381.2012.01994.x","article-title":"3-Nitropropionic acid induces autophagy by forming mitochondrial permeability transition pores rather than activating the mitochondrial fission pathway","volume":"168","author":"Solesio","year":"2013","journal-title":"Br. J. Pharmacol."},{"key":"key\n\t\t\t\t20181010112241222_ref48","doi-asserted-by":"crossref","first-page":"1760","DOI":"10.1101\/gr.135350.111","article-title":"GENCODE: the reference human genome annotation for The ENCODE Project","volume":"22","author":"Harrow","year":"2012","journal-title":"Genome Res."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/advance-article-pdf\/doi\/10.1093\/hmg\/ddy331\/25992964\/ddy331.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,10,24]],"date-time":"2019-10-24T04:47:00Z","timestamp":1571892420000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/advance-article\/doi\/10.1093\/hmg\/ddy331\/5103513"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9,19]]},"references-count":48,"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddy331","relation":{},"ISSN":["0964-6906","1460-2083"],"issn-type":[{"value":"0964-6906","type":"print"},{"value":"1460-2083","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9,19]]}}}