{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T18:10:18Z","timestamp":1790878218594,"version":"4.1.0"},"reference-count":87,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2025,5,1]],"date-time":"2025-05-01T00:00:00Z","timestamp":1746057600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,5,1]],"date-time":"2025-05-01T00:00:00Z","timestamp":1746057600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,3,28]],"date-time":"2025-03-28T00:00:00Z","timestamp":1743120000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000011","name":"Howard Hughes Medical Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000011","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Cell Reports"],"published-print":{"date-parts":[[2025,5]]},"DOI":"10.1016\/j.celrep.2025.115582","type":"journal-article","created":{"date-parts":[[2025,4,14]],"date-time":"2025-04-14T15:06:33Z","timestamp":1744643193000},"page":"115582","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":9,"title":["DM1 repeat-expanded RNAs confer RNA toxicity as individual nuclear-retained RNAs"],"prefix":"10.1016","volume":"44","author":[{"given":"John Paul Tsu","family":"Ouyang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siddharth","family":"Shukla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mona","family":"Bensalah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0486-0361","authenticated-orcid":false,"given":"Roy","family":"Parker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.celrep.2025.115582_bib1","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/B978-0-444-63233-3.00009-9","article-title":"Repeat expansion diseases","volume":"147","author":"Paulson","year":"2018","journal-title":"Handb. Clin. Neurol."},{"key":"10.1016\/j.celrep.2025.115582_bib2","doi-asserted-by":"crossref","DOI":"10.15252\/embj.2018101112","article-title":"RNA toxicity in non-coding repeat expansion disorders","volume":"39","author":"Swinnen","year":"2020","journal-title":"EMBO J."},{"key":"10.1016\/j.celrep.2025.115582_bib3","doi-asserted-by":"crossref","first-page":"4134","DOI":"10.1074\/jbc.REV119.007678","article-title":"On the wrong DNA track: Molecular mechanisms of repeat-mediated genome instability","volume":"295","author":"Khristich","year":"2020","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.celrep.2025.115582_bib4","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1038\/s41580-021-00382-6","article-title":"Molecular mechanisms underlying nucleotide repeat expansion disorders","volume":"22","author":"Malik","year":"2021","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.celrep.2025.115582_bib5","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1038\/s10038-022-01116-y","article-title":"Recent advances in CGG repeat diseases and a proposal of fragile X-associated tremor\/ataxia syndrome, neuronal intranuclear inclusion disease, and oculophryngodistal myopathy (FNOP) spectrum disorder","volume":"68","author":"Ishiura","year":"2023","journal-title":"J. Hum. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib6","doi-asserted-by":"crossref","first-page":"2433","DOI":"10.1093\/nar\/gkv029","article-title":"Molecular mechanisms in DM1 \u2014 a focus on foci","volume":"43","author":"Pettersson","year":"2015","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.celrep.2025.115582_bib7","doi-asserted-by":"crossref","first-page":"4439","DOI":"10.1093\/emboj\/19.17.4439","article-title":"Recruitment of human muscleblind proteins to (CUG)n expansions associated with myotonic dystrophy","volume":"19","author":"Miller","year":"2000","journal-title":"EMBO J."},{"key":"10.1016\/j.celrep.2025.115582_bib8","doi-asserted-by":"crossref","first-page":"R45","DOI":"10.1093\/hmg\/ddt371","article-title":"Repeat-associated non-ATG (RAN) translation in neurological disease","volume":"22","author":"Cleary","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib9","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1016\/j.conb.2021.11.001","article-title":"RAN proteins in neurodegenerative disease: repeating themes and unifying therapeutic strategies","volume":"72","author":"Guo","year":"2022","journal-title":"Curr. Opin. Neurobiol."},{"key":"10.1016\/j.celrep.2025.115582_bib10","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.gde.2017.01.005","article-title":"RNA toxicity and foci formation in microsatellite expansion diseases","volume":"44","author":"Zhang","year":"2017","journal-title":"Curr. Opin. Genet. Dev."},{"key":"10.1016\/j.celrep.2025.115582_bib11","doi-asserted-by":"crossref","first-page":"3811","DOI":"10.1093\/hmg\/ddr299","article-title":"Cellular toxicity of expanded RNA repeats: focus on RNA foci","volume":"20","author":"Wojciechowska","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib12","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1083\/jcb.128.6.995","article-title":"Foci of trinucleotide repeat transcripts in nuclei of myotonic dystrophy cells and tissues","volume":"128","author":"Taneja","year":"1995","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.celrep.2025.115582_bib13","doi-asserted-by":"crossref","first-page":"7388","DOI":"10.1073\/pnas.94.14.7388","article-title":"Expansion of a CUG trinucleotide repeat in the 3\u2032 untranslated region of myotonic dystrophy protein kinase transcripts results in nuclear retention of transcripts","volume":"94","author":"Davis","year":"1997","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2025.115582_bib14","doi-asserted-by":"crossref","first-page":"31079","DOI":"10.1074\/jbc.272.49.31079","article-title":"CUG Repeats Present in Myotonin Kinase RNA Form Metastable \u201cSlippery\u201d Hairpins","volume":"272","author":"Napiera\u0142a","year":"1997","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.celrep.2025.115582_bib15","doi-asserted-by":"crossref","first-page":"3534","DOI":"10.1093\/nar\/27.17.3534","article-title":"Visualization of double-stranded RNAs from the myotonic dystrophy protein kinase gene and interactions with CUG-binding protein","volume":"27","author":"Michalowski","year":"1999","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.celrep.2025.115582_bib16","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1017\/S1355838200991544","article-title":"Expanded CUG repeat RNAs form hairpins that activate the double-stranded RNA-dependent protein kinase PKR","volume":"6","author":"Tian","year":"2000","journal-title":"RNA"},{"key":"10.1016\/j.celrep.2025.115582_bib17","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1261\/rna.068940.118","article-title":"Expanded CUG repeats in DMPK transcripts adopt diverse hairpin conformations without influencing the structure of the flanking sequences","volume":"25","author":"van Cruchten","year":"2019","journal-title":"RNA"},{"key":"10.1016\/j.celrep.2025.115582_bib18","doi-asserted-by":"crossref","DOI":"10.3389\/fncel.2017.00097","article-title":"Structural characteristics of simple RNA repeats associated with disease and their deleterious protein interactions","volume":"11","author":"Ciesiolka","year":"2017","journal-title":"Front. Cell. Neurosci."},{"key":"10.1016\/j.celrep.2025.115582_bib19","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":"Nat"},{"key":"10.1016\/j.celrep.2025.115582_bib20","doi-asserted-by":"crossref","first-page":"5773","DOI":"10.1074\/jbc.M410781200","article-title":"MBNL1 is the primary determinant of focus formation and aberrant insulin receptor splicing in DM1","volume":"280","author":"Dansithong","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.celrep.2025.115582_bib21","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1093\/hmg\/ddu492","article-title":"Nuclear localization of MBNL1: splicing-mediated autoregulation and repression of repeat-derived aberrant proteins","volume":"24","author":"Kino","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib22","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.ymeth.2017.04.002","article-title":"2D and 3D FISH of expanded repeat RNAs in human lymphoblasts","volume":"120","author":"Urbanek","year":"2017","journal-title":"Methods"},{"key":"10.1016\/j.celrep.2025.115582_bib23","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1242\/jcs.073270","article-title":"Stochastic and reversible aggregation of mRNA with expanded CUG-triplet repeats","volume":"124","author":"Querido","year":"2011","journal-title":"J. Cell Sci."},{"key":"10.1016\/j.celrep.2025.115582_bib24","doi-asserted-by":"crossref","first-page":"1975","DOI":"10.1093\/hmg\/8.11.1975","article-title":"Cis and Trans Effects of the Myotonic Dystrophy (DM) Mutation in a Cell Culture Model","volume":"8","author":"Amack","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib25","doi-asserted-by":"crossref","first-page":"1879","DOI":"10.1093\/hmg\/10.18.1879","article-title":"The myotonic dystrophy expanded CUG repeat tract is necessary but not sufficient to disrupt C2C12 myoblast differentiation","volume":"10","author":"Amack","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib26","doi-asserted-by":"crossref","first-page":"4221","DOI":"10.1073\/pnas.1117019109","article-title":"RNase H-mediated degradation of toxic RNA in myotonic dystrophy type 1","volume":"109","author":"Lee","year":"2012","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2025.115582_bib27","doi-asserted-by":"crossref","first-page":"8041","DOI":"10.1073\/pnas.1422273112","article-title":"Reduction of toxic RNAs in myotonic dystrophies type 1 and type 2 by the RNA helicase p68\/DDX5","volume":"112","author":"Jones","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2025.115582_bib28","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.85902","article-title":"Antisense, but not sense, repeat expanded RNAs activate PKR\/eIF2\u03b1-dependent ISR in C9ORF72 FTD\/ALS","volume":"12","author":"Parameswaran","year":"2023","journal-title":"Elife"},{"key":"10.1016\/j.celrep.2025.115582_bib29","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/j.chembiol.2016.12.018","article-title":"c9orf72 Disease-Related Foci Are Each Composed of One Mutant Expanded Repeat RNA","volume":"24","author":"Liu","year":"2017","journal-title":"Cell Chem. Biol."},{"key":"10.1016\/j.celrep.2025.115582_bib30","doi-asserted-by":"crossref","first-page":"1648","DOI":"10.1093\/hmg\/ddw042","article-title":"A low absolute number of expanded transcripts is involved in myotonic dystrophy type 1 manifestation in muscle","volume":"25","author":"Gudde","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib31","doi-asserted-by":"crossref","first-page":"5885","DOI":"10.1038\/s41598-018-24156-x","article-title":"Quantitative Methods to Monitor RNA Biomarkers in Myotonic Dystrophy","volume":"8","author":"Wojciechowska","year":"2018","journal-title":"Sci. Rep."},{"key":"10.1016\/j.celrep.2025.115582_bib32","doi-asserted-by":"crossref","first-page":"799","DOI":"10.1016\/0092-8674(92)90154-5","article-title":"Molecular basis of myotonic dystrophy: Expansion of a trinucleotide (CTG) repeat at the 3\u2032 end of a transcript encoding a protein kinase family member","volume":"68","author":"Brook","year":"1992","journal-title":"Cell"},{"key":"10.1016\/j.celrep.2025.115582_bib33","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1126\/science.1546325","article-title":"Myotonic Dystrophy Mutation: an Unstable CTG Repeat in the 3\u2032 Untranslated region of the Gene","volume":"255","author":"Mahadevan","year":"1992","journal-title":"Science"},{"key":"10.1016\/j.celrep.2025.115582_bib34","doi-asserted-by":"crossref","first-page":"1256","DOI":"10.1126\/science.1546326","article-title":"An Unstable Triplet Repeat in a Gene Related to Myotonic Muscular Dystrophy","volume":"255","author":"Fu","year":"1992","journal-title":"Science"},{"key":"10.1016\/j.celrep.2025.115582_bib35","first-page":"487","article-title":"Immortalized human myotonic dystrophy muscle cell lines to assess therapeutic compounds","volume":"10","author":"Arandel","year":"2017","journal-title":"Dis. Model. Mech."},{"key":"10.1016\/j.celrep.2025.115582_bib36","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1038\/s41419-018-1080-1","article-title":"Activation of the interferon type I response rather than autophagy contributes to myogenesis inhibition in congenital DM1 myoblasts","volume":"9","author":"Rizzo","year":"2018","journal-title":"Cell Death Dis."},{"key":"10.1016\/j.celrep.2025.115582_bib37","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1083\/jcb.200206020","article-title":"Mutant DMPK 3\u2032-UTR transcripts disrupt C2C12 myogenic differentiation by compromising MyoD","volume":"159","author":"Amack","year":"2002","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.celrep.2025.115582_bib38","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1007\/978-1-4939-9784-8_13","article-title":"FISH Protocol for Myotonic Dystrophy Type 1 Cells","volume":"2056","author":"Klein","year":"2020","journal-title":"Methods Mol. Biol."},{"key":"10.1016\/j.celrep.2025.115582_bib39","doi-asserted-by":"crossref","DOI":"10.1093\/nar\/gkw784","article-title":"smiFISH and FISH-quant \u2013 a flexible single RNA detection approach with super-resolution capability","volume":"44","author":"Tsanov","year":"2016","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.celrep.2025.115582_bib40","doi-asserted-by":"crossref","first-page":"1840","DOI":"10.1093\/bioinformatics\/btt276","article-title":"TANGO: a generic tool for high-throughput 3D image analysis for studying nuclear organization","volume":"29","author":"Ollion","year":"2013","journal-title":"Bioinformatics"},{"key":"10.1016\/j.celrep.2025.115582_bib41","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1126\/science.1242939","article-title":"Single \u03b2-actin mRNA detection in neurons reveals a mechanism for regulating its translatability","volume":"343","author":"Buxbaum","year":"2014","journal-title":"Science"},{"key":"10.1016\/j.celrep.2025.115582_bib42","doi-asserted-by":"crossref","first-page":"2766","DOI":"10.1093\/nar\/29.13.2766","article-title":"In vivo co-localisation of MBNL protein with DMPK expanded-repeat transcripts","volume":"29","author":"Fardaei","year":"2001","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.celrep.2025.115582_bib43","doi-asserted-by":"crossref","first-page":"2165","DOI":"10.1093\/hmg\/10.19.2165","article-title":"Muscleblind localizes to nuclear foci of aberrant RNA in myotonic dystrophy types 1 and 2","volume":"10","author":"Mankodi","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib44","doi-asserted-by":"crossref","DOI":"10.1212\/NXG.0000000000000484","article-title":"Three-dimensional imaging in myotonic dystrophy type 1: Linking molecular alterations with disease phenotype","volume":"6","author":"Ballester-Lopez","year":"2020","journal-title":"Neurol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib45","doi-asserted-by":"crossref","first-page":"764","DOI":"10.1016\/j.tibs.2020.05.002","article-title":"Mechanisms and Regulation of RNA Condensation in RNP Granule Formation","volume":"45","author":"Tauber","year":"2020","journal-title":"Trends Biochem. Sci."},{"key":"10.1016\/j.celrep.2025.115582_bib46","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1101\/gad.350518.123","article-title":"RNA granules: functional compartments or incidental condensates?","volume":"37","author":"Putnam","year":"2023","journal-title":"Genes Dev."},{"key":"10.1016\/j.celrep.2025.115582_bib47","doi-asserted-by":"crossref","first-page":"4737","DOI":"10.1016\/j.cell.2023.09.006","article-title":"Formation, function, and pathology of RNP granules","volume":"186","author":"Ripin","year":"2023","journal-title":"Cell"},{"key":"10.1016\/j.celrep.2025.115582_bib48","doi-asserted-by":"crossref","first-page":"3103","DOI":"10.1038\/sj.emboj.7600300","article-title":"Muscleblind proteins regulate alternative splicing","volume":"23","author":"Ho","year":"2004","journal-title":"EMBO J."},{"key":"10.1016\/j.celrep.2025.115582_bib49","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1038\/s41551-021-00838-2","article-title":"Reversal of RNA toxicity in myotonic dystrophy via a decoy RNA-binding protein with high affinity for expanded CUG repeats","volume":"6","author":"Arandel","year":"2021","journal-title":"Nat. Biomed. Eng."},{"key":"10.1016\/j.celrep.2025.115582_bib50","doi-asserted-by":"crossref","first-page":"3427","DOI":"10.1038\/s41467-023-38923-6","article-title":"Muscleblind-like proteins use modular domains to localize RNAs by riding kinesins and docking to membranes","volume":"14","author":"Hildebrandt","year":"2023","journal-title":"Nat. Commun."},{"key":"10.1016\/j.celrep.2025.115582_bib51","doi-asserted-by":"crossref","first-page":"2646","DOI":"10.1073\/pnas.0708519105","article-title":"Expanded CTG repeats within the DMPK 3\u2032 UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophy","volume":"105","author":"Orengo","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2025.115582_bib52","doi-asserted-by":"crossref","first-page":"2802","DOI":"10.1172\/JCI32308","article-title":"Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy","volume":"117","author":"Wang","year":"2007","journal-title":"J. Clin. Investig."},{"key":"10.1016\/j.celrep.2025.115582_bib53","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1038\/ncomms1103","article-title":"A role for TREX components in the release of spliced mRNA from nuclear speckle domains","volume":"1","author":"Dias","year":"2010","journal-title":"Nat. Commun."},{"key":"10.1016\/j.celrep.2025.115582_bib54","doi-asserted-by":"crossref","first-page":"15001","DOI":"10.1038\/celldisc.2015.1","article-title":"Premature termination codons are recognized in the nucleus in a reading-frame-dependent manner","volume":"1","author":"Shi","year":"2015","journal-title":"Cell Discov."},{"key":"10.1016\/j.celrep.2025.115582_bib55","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0124958","article-title":"MXS-Chaining: A Highly Efficient Cloning Platform for Imaging and Flow Cytometry Approaches in Mammalian Systems","volume":"10","author":"Sladitschek","year":"2015","journal-title":"PLoS One"},{"key":"10.1016\/j.celrep.2025.115582_bib56","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1126\/science.1070919","article-title":"Stochastic gene expression in a single cell","volume":"297","author":"Elowitz","year":"2002","journal-title":"Science"},{"key":"10.1016\/j.celrep.2025.115582_bib57","doi-asserted-by":"crossref","first-page":"1811","DOI":"10.1126\/science.1098641","article-title":"Control of stochasticity in eukaryotic gene expression","volume":"304","author":"Raser","year":"2004","journal-title":"Science"},{"key":"10.1016\/j.celrep.2025.115582_bib58","doi-asserted-by":"crossref","first-page":"1188","DOI":"10.1126\/science.1242975","article-title":"Genetic determinants and cellular constraints in noisy gene expression","volume":"342","author":"Sanchez","year":"2013","journal-title":"Science"},{"key":"10.1016\/j.celrep.2025.115582_bib59","doi-asserted-by":"crossref","first-page":"1263","DOI":"10.1038\/nsmb.1514","article-title":"Single-RNA counting reveals alternative modes of gene expression in yeast","volume":"15","author":"Zenklusen","year":"2008","journal-title":"Nat. Struct. Mol. Biol."},{"key":"10.1016\/j.celrep.2025.115582_bib60","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1038\/nature01546","article-title":"Noise in eukaryotic gene expression","volume":"422","author":"Blake","year":"2003","journal-title":"Nature"},{"key":"10.1016\/j.celrep.2025.115582_bib61","doi-asserted-by":"crossref","first-page":"2565","DOI":"10.1242\/jcs.108.7.2565","article-title":"RNAs radiate from gene to cytoplasm as revealed by fluorescence in situ hybridization","volume":"108","author":"Dirks","year":"1995","journal-title":"J. Cell Sci."},{"key":"10.1016\/j.celrep.2025.115582_bib62","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1006\/excr.1997.3838","article-title":"Contribution of Growing RNA Molecules to the Nuclear Transcripts Foci Observed by FISH","volume":"238","author":"Jolly","year":"1998","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.celrep.2025.115582_bib63","doi-asserted-by":"crossref","first-page":"2855","DOI":"10.1093\/emboj\/18.10.2855","article-title":"Inefficient processing impairs release of RNA from the site of transcription","volume":"18","author":"Cust\u00f3dio","year":"1999","journal-title":"EMBO J."},{"key":"10.1016\/j.celrep.2025.115582_bib64","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/S1097-2765(01)00288-X","article-title":"Precursor RNAs harboring nonsense codons accumulate near the site of transcription","volume":"8","author":"M\u00fchlemann","year":"2001","journal-title":"Mol. Cell"},{"key":"10.1016\/j.celrep.2025.115582_bib65","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1038\/s41556-022-00940-w","article-title":"The conserved helicase ZNFX-1 memorializes silenced RNAs in perinuclear condensates","volume":"24","author":"Ouyang","year":"2022","journal-title":"Nat. Cell Biol."},{"key":"10.1016\/j.celrep.2025.115582_bib66","doi-asserted-by":"crossref","first-page":"1207","DOI":"10.1016\/j.neuron.2015.09.015","article-title":"Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD\/ALS","volume":"87","author":"Tran","year":"2015","journal-title":"Neuron"},{"key":"10.1016\/j.celrep.2025.115582_bib67","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1038\/nmeth.1253","article-title":"Imaging individual mRNA molecules using multiple singly labeled probes","volume":"5","author":"Raj","year":"2008","journal-title":"Nat. Methods"},{"key":"10.1016\/j.celrep.2025.115582_bib68","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1126\/science.280.5363.585","article-title":"Visualization of single RNA transcripts in situ","volume":"280","author":"Femino","year":"1998","journal-title":"Science"},{"key":"10.1016\/j.celrep.2025.115582_bib71","doi-asserted-by":"crossref","first-page":"11748","DOI":"10.1073\/pnas.0604970103","article-title":"Reversal of RNA missplicing and myotonia after muscleblind overexpression in a mouse poly(CUG) model for myotonic dystrophy","volume":"103","author":"Kanadia","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.celrep.2025.115582_bib72","doi-asserted-by":"crossref","first-page":"4645","DOI":"10.1093\/hmg\/dds306","article-title":"Mouse model of muscleblind-like 1 overexpression: skeletal muscle effects and therapeutic promise","volume":"21","author":"Chamberlain","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib73","doi-asserted-by":"crossref","first-page":"1978","DOI":"10.1126\/science.1088583","article-title":"A Muscleblind Knockout Model for Myotonic Dystrophy","volume":"302","author":"Kanadia","year":"2003","journal-title":"Science"},{"key":"10.1016\/j.celrep.2025.115582_bib74","doi-asserted-by":"crossref","first-page":"4350","DOI":"10.1074\/jbc.M116.770768","article-title":"Pseudouridine Modification Inhibits Muscleblind-like 1 (MBNL1) Binding to CCUG Repeats and Minimally Structured RNA through Reduced RNA Flexibility","volume":"292","author":"Delorimier","year":"2017","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.celrep.2025.115582_bib75","doi-asserted-by":"crossref","first-page":"2467","DOI":"10.1093\/nar\/gkp1209","article-title":"MBNL1 binds GC motifs embedded in pyrimidines to regulate alternative splicing","volume":"38","author":"Goers","year":"2010","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.celrep.2025.115582_bib76","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.16950","article-title":"Global, quantitative and dynamic mapping of protein subcellular localization","volume":"5","author":"Itzhak","year":"2016","journal-title":"Elife"},{"key":"10.1016\/j.celrep.2025.115582_bib77","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1093\/hmg\/11.7.805","article-title":"Three proteins, MBNL, MBLL and MBXL, co-localize in vivo with nuclear foci of expanded-repeat transcripts in DM1 and DM2 cells","volume":"11","author":"Fardaei","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib78","doi-asserted-by":"crossref","first-page":"1159","DOI":"10.1016\/j.celrep.2015.07.029","article-title":"MBNL Sequestration by Toxic RNAs and RNA Mis-Processing in the Myotonic Dystrophy Brain","volume":"12","author":"Goodwin","year":"2015","journal-title":"Cell Rep."},{"key":"10.1016\/j.celrep.2025.115582_bib79","doi-asserted-by":"crossref","first-page":"2923","DOI":"10.1242\/jcs.02404","article-title":"Colocalization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophy","volume":"118","author":"Ho","year":"2005","journal-title":"J. Cell Sci."},{"key":"10.1016\/j.celrep.2025.115582_bib80","doi-asserted-by":"crossref","first-page":"1165","DOI":"10.1038\/s41586-024-07429-6","article-title":"Genome organization around nuclear speckles drives mRNA splicing efficiency","volume":"629","author":"Bhat","year":"2024","journal-title":"Nature"},{"key":"10.1016\/j.celrep.2025.115582_bib81","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1093\/hmg\/ddr515","article-title":"Activation of the innate immune response and interferon signalling in myotonic dystrophy type 1 and type 2 cataracts","volume":"21","author":"Rhodes","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib82","doi-asserted-by":"crossref","first-page":"3706","DOI":"10.1096\/fj.09-151159","article-title":"Expansion of CUG RNA repeats causes stress and inhibition of translation in myotonic dystrophy 1 (DM1) cells","volume":"24","author":"Huichalaf","year":"2010","journal-title":"FASEB J."},{"key":"10.1016\/j.celrep.2025.115582_bib83","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1016\/j.molcel.2007.01.031","article-title":"Ribonuclease Dicer Cleaves Triplet Repeat Hairpins into Shorter Repeats that Silence Specific Targets","volume":"25","author":"Krol","year":"2007","journal-title":"Mol. Cell"},{"key":"10.1016\/j.celrep.2025.115582_bib84","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1001340","article-title":"Triplet Repeat\u2013Derived siRNAs Enhance RNA\u2013Mediated Toxicity in a Drosophila Model for Myotonic Dystrophy","volume":"7","author":"Yu","year":"2011","journal-title":"PLoS Genet."},{"key":"10.1016\/j.celrep.2025.115582_bib85","doi-asserted-by":"crossref","first-page":"1711","DOI":"10.1016\/j.jmb.2019.03.003","article-title":"Expanded CUG Repeats Trigger Disease Phenotype and Expression Changes through the RNAi Machinery in C. elegans","volume":"431","author":"Qawasmi","year":"2019","journal-title":"J. Mol. Biol."},{"key":"10.1016\/j.celrep.2025.115582_bib87","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1038\/nmeth.2019","article-title":"Fiji: an open-source platform for biological-image analysis","volume":"9","author":"Schindelin","year":"2012","journal-title":"Nat. Methods"},{"key":"10.1016\/j.celrep.2025.115582_bib88","doi-asserted-by":"crossref","DOI":"10.7717\/peerj.453","article-title":"Scikit-image: Image processing in python","volume":"2","author":"Van Der Walt","year":"2014","journal-title":"PeerJ"},{"key":"10.1016\/j.celrep.2025.115582_bib89","series-title":"ggplot2","author":"Wickham","year":"2016"},{"key":"10.1016\/j.celrep.2025.115582_bib90","doi-asserted-by":"crossref","first-page":"1582","DOI":"10.1261\/rna.061184.117","article-title":"Enzymatic production of single-molecule FISH and RNA capture probes","volume":"23","author":"Gaspar","year":"2017","journal-title":"RNA"}],"container-title":["Cell Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2211124725003535?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2211124725003535?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T05:37:35Z","timestamp":1776836255000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2211124725003535"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5]]},"references-count":87,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2025,5]]}},"alternative-id":["S2211124725003535"],"URL":"https:\/\/doi.org\/10.1016\/j.celrep.2025.115582","relation":{},"ISSN":["2211-1247"],"issn-type":[{"value":"2211-1247","type":"print"}],"subject":[],"published":{"date-parts":[[2025,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"DM1 repeat-expanded RNAs confer RNA toxicity as individual nuclear-retained RNAs","name":"articletitle","label":"Article Title"},{"value":"Cell Reports","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.celrep.2025.115582","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 The Author(s). Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"115582"}}