{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,14]],"date-time":"2026-06-14T21:51:24Z","timestamp":1781473884715,"version":"3.54.1"},"reference-count":108,"publisher":"Oxford University Press (OUP)","issue":"19","license":[{"start":{"date-parts":[[2024,9,11]],"date-time":"2024-09-11T00:00:00Z","timestamp":1726012800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000780","name":"European Union","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008530","name":"European Regional Development Fund","doi-asserted-by":"publisher","award":["POIR.04.04.00-00-5C0C\/17-00"],"award-info":[{"award-number":["POIR.04.04.00-00-5C0C\/17-00"]}],"id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004281","name":"National Science Centre","doi-asserted-by":"publisher","award":["2014\/15\/B\/NZ2\/02453"],"award-info":[{"award-number":["2014\/15\/B\/NZ2\/02453"]}],"id":[{"id":"10.13039\/501100004281","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004281","name":"National Science Centre","doi-asserted-by":"publisher","award":["2020\/38\/A\/NZ3\/00498"],"award-info":[{"award-number":["2020\/38\/A\/NZ3\/00498"]}],"id":[{"id":"10.13039\/501100004281","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004281","name":"National Science Centre","doi-asserted-by":"publisher","award":["2019\/03\/X\/NZ1\/01164"],"award-info":[{"award-number":["2019\/03\/X\/NZ1\/01164"]}],"id":[{"id":"10.13039\/501100004281","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Initiative of Excellence\u2013Research University at Adam Mickiewicz University, Poznan, Poland"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,28]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Muscleblind like splicing regulators (MBNLs) govern various RNA-processing steps, including alternative splicing, polyadenylation, RNA stability\u00a0and mRNA intracellular localization. In myotonic dystrophy type 1 (DM1), the most common muscular dystrophy in adults, MBNLs are sequestered on toxic RNA containing expanded CUG repeats, which leads to disruption of MBNL-regulated processes and disease features of DM1. Herein, we show the significance of MBNLs in regulating microtranscriptome dynamics during the postnatal development of skeletal muscles and in microRNA (miRNA) misregulation observed in mouse models and patients with DM1. We identify multiple miRNAs sensitive to MBNL proteins insufficiency and reveal that many of them were postnatally regulated, which correlates with increases in the activity of these proteins during this process. In adult Mbnl1-knockout mice, miRNA expression exhibited an adult-to-newborn shift. We hypothesize that Mbnl1 deficiency influences miRNA levels through a combination of mechanisms. First, the absence of Mbnl1 protein results in alterations to the levels of pri-miRNAs. Second, MBNLs affect miRNA biogenesis by regulating the alternative splicing of miRNA primary transcripts. We propose that the expression of miR-23b, miR-27b and miR-24-1, produced from the same cluster, depends on the MBNL-sensitive inclusion of alternative exons containing miRNA sequences. Our findings suggest that MBNL sequestration in DM1 is partially responsible for altered miRNA activity. This study provides new insights into the biological roles and functions of MBNL proteins as regulators of miRNA expression in skeletal muscles.<\/jats:p>","DOI":"10.1093\/nar\/gkae774","type":"journal-article","created":{"date-parts":[[2024,9,11]],"date-time":"2024-09-11T11:04:39Z","timestamp":1726052679000},"page":"12055-12073","source":"Crossref","is-referenced-by-count":13,"title":["MBNL splicing factors regulate the microtranscriptome of skeletal muscles"],"prefix":"10.1093","volume":"52","author":[{"given":"Agnieszka","family":"Piasecka","sequence":"first","affiliation":[{"name":"Laboratory of Gene Therapy, Department of Gene Expression, Institute of Molecular Biology and Biotechnology, Faculty of Biology, Adam Mickiewicz University , Uniwersytetu Poznanskiego 6, 61-614 Pozna\u0144,","place":["Poland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Micha\u0142\u00a0W","family":"Szcze\u015bniak","sequence":"additional","affiliation":[{"name":"Institute of Human Biology and Evolution, Faculty of Biology, Adam Mickiewicz University , Uniwersytetu Poznanskiego 6, 61-614 Pozna\u0144,","place":["Poland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Micha\u0142","family":"Sekrecki","sequence":"additional","affiliation":[{"name":"Laboratory of Gene Therapy, Department of Gene Expression, Institute of Molecular Biology and Biotechnology, Faculty of Biology, Adam Mickiewicz University , Uniwersytetu Poznanskiego 6, 61-614 Pozna\u0144,","place":["Poland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arkadiusz","family":"Kajdasz","sequence":"additional","affiliation":[{"name":"Laboratory of Gene Therapy, Department of Gene Expression, Institute of Molecular Biology and Biotechnology, Faculty of Biology, Adam Mickiewicz University , Uniwersytetu Poznanskiego 6, 61-614 Pozna\u0144,","place":["Poland"]},{"name":"Laboratory of Bioinformatics, Institute of Bioorganic Chemistry, Polish Academy of Sciences , Noskowskiego 12\/14, 61-704 Pozna\u0144 ,","place":["Poland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u0141ukasz\u00a0J","family":"Sznajder","sequence":"additional","affiliation":[{"name":"Laboratory of Gene Therapy, Department of Gene Expression, Institute of Molecular Biology and Biotechnology, Faculty of Biology, Adam Mickiewicz University , Uniwersytetu Poznanskiego 6, 61-614 Pozna\u0144,","place":["Poland"]},{"name":"Department of Chemistry and Biochemistry, University of Nevada , Las Vegas , NV 89154 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Baud","sequence":"additional","affiliation":[{"name":"Laboratory of Gene Therapy, Department of Gene Expression, Institute of Molecular Biology and Biotechnology, Faculty of Biology, Adam Mickiewicz University , Uniwersytetu Poznanskiego 6, 61-614 Pozna\u0144,","place":["Poland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8352-9812","authenticated-orcid":false,"given":"Krzysztof","family":"Sobczak","sequence":"additional","affiliation":[{"name":"Laboratory of Gene Therapy, Department of Gene Expression, Institute of Molecular Biology and Biotechnology, Faculty of Biology, Adam Mickiewicz University , Uniwersytetu Poznanskiego 6, 61-614 Pozna\u0144,","place":["Poland"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,9,11]]},"reference":[{"key":"2024102814473167100_B1","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1111\/j.1432-0436.2006.00060.x","article-title":"The Muscleblind family of proteins: an emerging class of regulators of developmentally programmed alternative splicing","volume":"74","author":"Pascual","year":"2006","journal-title":"Differentiation"},{"key":"2024102814473167100_B2","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1016\/j.molcel.2014.08.027","article-title":"Loss of MBNL leads to disruption of developmentally regulated alternative polyadenylation in RNA-mediated disease","volume":"56","author":"Batra","year":"2014","journal-title":"Mol. Cell"},{"key":"2024102814473167100_B3","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1016\/j.cell.2012.06.041","article-title":"Transcriptome-wide regulation of pre-mRNA splicing and mRNA localization by muscleblind proteins","volume":"150","author":"Wang","year":"2012","journal-title":"Cell"},{"key":"2024102814473167100_B4","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1038\/srep00209","article-title":"CUGBP1 and MBNL1 preferentially bind to 3\u2019 UTRs and facilitate mRNA decay","volume":"2","author":"Masuda","year":"2012","journal-title":"Sci. Rep."},{"key":"2024102814473167100_B5","doi-asserted-by":"crossref","first-page":"1281","DOI":"10.1042\/BST0371281","article-title":"Pathogenic mechanisms of myotonic dystrophy","volume":"37","author":"Lee","year":"2009","journal-title":"Biochem. Soc. Trans."},{"key":"2024102814473167100_B6","doi-asserted-by":"crossref","first-page":"2280","DOI":"10.1016\/j.biocel.2013.06.010","article-title":"Molecular mechanisms of muscle atrophy in myotonic dystrophies","volume":"45","author":"Timchenko","year":"2013","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"2024102814473167100_B7","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":"2024102814473167100_B8","doi-asserted-by":"crossref","first-page":"43","DOI":"10.4161\/rna.7.1.10401","article-title":"Conserved developmental alternative splicing of muscleblind-like (MBNL) transcripts regulates MBNL localization and activity","volume":"7","author":"Terenzi","year":"2010","journal-title":"RNA Biol."},{"key":"2024102814473167100_B9","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":"2024102814473167100_B10","doi-asserted-by":"crossref","first-page":"2087","DOI":"10.1093\/hmg\/ddl132","article-title":"Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy","volume":"15","author":"Lin","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"2024102814473167100_B11","doi-asserted-by":"crossref","first-page":"20333","DOI":"10.1073\/pnas.0809045105","article-title":"A postnatal switch of CELF and MBNL proteins reprograms alternative splicing in the developing heart","volume":"105","author":"Kalsotra","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2024102814473167100_B12","doi-asserted-by":"crossref","first-page":"e27192","DOI":"10.7554\/eLife.27192","article-title":"Extensive alternative splicing transitions during postnatal skeletal muscle development are required for calcium handling functions","volume":"6","author":"Brinegar","year":"2017","journal-title":"eLife"},{"key":"2024102814473167100_B13","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1016\/j.neuron.2012.05.029","article-title":"Muscleblind-like 2-mediated alternative splicing in the developing brain and dysregulation in myotonic dystrophy","volume":"75","author":"Charizanis","year":"2012","journal-title":"Neuron"},{"key":"2024102814473167100_B14","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":"2024102814473167100_B15","doi-asserted-by":"crossref","first-page":"e3001615","DOI":"10.1371\/journal.pbio.3001615","article-title":"The X-linked splicing regulator MBNL3 has been co-opted to restrict placental growth in eutherians","volume":"20","author":"Spruce","year":"2022","journal-title":"PLoS Biol."},{"key":"2024102814473167100_B16","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":"2024102814473167100_B17","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":"2024102814473167100_B18","first-page":"3952","article-title":"Correction of ClC-1 splicing eliminates chloride channelopathy and myotonia in mouse models of myotonic dystrophy","volume":"117","author":"Wheeler","year":"2007","journal-title":"J. Clin. Invest."},{"key":"2024102814473167100_B19","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1002\/cphy.c170002","article-title":"Myotonic dystrophy and developmental regulation of RNA processing","volume-title":"Comprehensive Physiology","author":"Thomas","year":"2018"},{"key":"2024102814473167100_B20","doi-asserted-by":"crossref","first-page":"9042","DOI":"10.1038\/srep09042","article-title":"Loss of muscleblind-like 1 results in cardiac pathology and persistence of embryonic splice isoforms","volume":"5","author":"Dixon","year":"2015","journal-title":"Sci. Rep."},{"key":"2024102814473167100_B21","doi-asserted-by":"crossref","first-page":"3144","DOI":"10.1093\/hmg\/ddac108","article-title":"Mice lacking MBNL1 and MBNL2 exhibit sudden cardiac death and molecular signatures recapitulating myotonic dystrophy","volume":"31","author":"Lee","year":"2022","journal-title":"Hum. Mol. Genet."},{"key":"2024102814473167100_B22","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1016\/j.nmd.2004.09.012","article-title":"RNA pathogenesis of the myotonic dystrophies","volume":"15","author":"Day","year":"2005","journal-title":"Neuromuscul. Disord."},{"key":"2024102814473167100_B23","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1007\/978-3-319-89689-2_8","article-title":"Deregulation of RNA metabolism in microsatellite expansion diseases","volume":"20","author":"Misra","year":"2018","journal-title":"Adv Neurobiol."},{"key":"2024102814473167100_B24","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1186\/1479-5876-8-48","article-title":"Overexpression of microRNA-206 in the skeletal muscle from myotonic dystrophy type 1 patients","volume":"8","author":"Gambardella","year":"2010","journal-title":"J. Transl. Med."},{"key":"2024102814473167100_B25","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.nmd.2010.11.012","article-title":"Dysregulation and cellular mislocalization of specific miRNAs in myotonic dystrophy type 1","volume":"21","author":"Perbellini","year":"2011","journal-title":"Neuromuscul. Disord."},{"key":"2024102814473167100_B26","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1093\/hmg\/dds478","article-title":"Expanded CTG repeats trigger miRNA alterations in Drosophila that are conserved in myotonic dystrophy type 1 patients","volume":"22","author":"Fernandez-Costa","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"2024102814473167100_B27","doi-asserted-by":"crossref","first-page":"38174","DOI":"10.1038\/srep38174","article-title":"Validation of plasma microRNAs as biomarkers for myotonic dystrophy type 1","volume":"6","author":"Perfetti","year":"2016","journal-title":"Sci. Rep."},{"key":"2024102814473167100_B28","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1038\/nrm3838","article-title":"Regulation of microRNA biogenesis","volume":"15","author":"Ha","year":"2014","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"2024102814473167100_B29","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1101\/gr.198788.115","article-title":"Degradation dynamics of microRNAs revealed by a novel pulse-chase approach","volume":"26","author":"Marzi","year":"2016","journal-title":"Genome Res."},{"key":"2024102814473167100_B30","doi-asserted-by":"crossref","first-page":"1777","DOI":"10.1101\/gr.251421.119","article-title":"Global analyses of the dynamics of mammalian microRNA metabolism","volume":"29","author":"Kingston","year":"2019","journal-title":"Genome Res."},{"key":"2024102814473167100_B31","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1016\/j.molcel.2019.06.018","article-title":"Time-resolved small RNA sequencing unravels the molecular principles of MicroRNA homeostasis","volume":"75","author":"Reichholf","year":"2019","journal-title":"Mol. Cell"},{"key":"2024102814473167100_B32","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1261\/rna.1155108","article-title":"Lin-28 interaction with the let-7 precursor loop mediates regulated microRNA processing","volume":"14","author":"Newman","year":"2008","journal-title":"RNA"},{"key":"2024102814473167100_B33","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1038\/ncb1759","article-title":"A feedback loop comprising lin-28 and let-7 controls pre-let-7 maturation during neural stem-cell commitment","volume":"10","author":"Rybak","year":"2008","journal-title":"Nat. Cell Biol."},{"key":"2024102814473167100_B34","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1016\/j.molcel.2008.09.014","article-title":"Lin28 Mediates the terminal uridylation of let-7 precursor MicroRNA","volume":"32","author":"Heo","year":"2008","journal-title":"Mol. Cell"},{"key":"2024102814473167100_B35","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1126\/science.1154040","article-title":"Selective blockade of MicroRNA processing by Lin28","volume":"320","author":"Viswanathan","year":"2008","journal-title":"Science"},{"key":"2024102814473167100_B36","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1038\/nature07086","article-title":"SMAD proteins control DROSHA-mediated microRNA maturation","volume":"454","author":"Davis","year":"2008","journal-title":"Nature"},{"key":"2024102814473167100_B37","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1038\/nature08199","article-title":"Modulation of microRNA processing by p53","volume":"460","author":"Suzuki","year":"2009","journal-title":"Nature"},{"key":"2024102814473167100_B38","doi-asserted-by":"crossref","first-page":"1010","DOI":"10.1038\/nature08025","article-title":"The RNA-binding protein KSRP promotes the biogenesis of a subset of microRNAs","volume":"459","author":"Trabucchi","year":"2009","journal-title":"Nature"},{"key":"2024102814473167100_B39","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1038\/nsmb1250","article-title":"The multifunctional RNA-binding protein hnRNP A1 is required for processing of miR-18a","volume":"14","author":"Guil","year":"2007","journal-title":"Nat. Struct. Mol. Biol."},{"key":"2024102814473167100_B40","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1016\/j.molcel.2017.03.014","article-title":"A compendium of RNA-binding proteins that regulate MicroRNA biogenesis","volume":"66","author":"Treiber","year":"2017","journal-title":"Mol. Cell"},{"key":"2024102814473167100_B41","doi-asserted-by":"crossref","first-page":"6610","DOI":"10.1093\/nar\/gkq547","article-title":"Phosphorylation of the RNase III enzyme drosha at Serine300 or Serine302 is required for its nuclear localization","volume":"38","author":"Tang","year":"2010","journal-title":"Nucleic Acids Res."},{"key":"2024102814473167100_B42","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1016\/j.cell.2009.06.044","article-title":"Phosphorylation of the Human MicroRNA-generating complex mediates MAPK\/erk signaling","volume":"139","author":"Paroo","year":"2009","journal-title":"Cell"},{"key":"2024102814473167100_B43","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1016\/j.celrep.2013.10.017","article-title":"Phosphorylation of DGCR8 increases its intracellular stability and induces a progrowth miRNA profile","volume":"5","author":"Herbert","year":"2013","journal-title":"Cell Rep."},{"key":"2024102814473167100_B44","doi-asserted-by":"crossref","first-page":"2100","DOI":"10.1016\/j.celrep.2013.05.038","article-title":"DNA-damage-induced nuclear export of precursor MicroRNAs is regulated by the ATM-AKT pathway","volume":"3","author":"Wan","year":"2013","journal-title":"Cell Rep."},{"key":"2024102814473167100_B45","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1038\/embor.2011.247","article-title":"Histone deacetylase 1 enhances microRNA processing via deacetylation of DGCR8","volume":"13","author":"Wada","year":"2012","journal-title":"EMBO Rep."},{"key":"2024102814473167100_B46","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1261\/rna.7240905","article-title":"Microarray profiling of microRNAs reveals frequent coexpression with neighboring miRNAs and host genes","volume":"11","author":"BASKERVILLE","year":"2005","journal-title":"RNA"},{"key":"2024102814473167100_B47","first-page":"166","article-title":"Characterization of microRNA expression profiles in normal human tissues","volume":"8","author":"Liang","year":"2007","journal-title":"Bmc Genomics [Electronic Resource]"},{"key":"2024102814473167100_B48","doi-asserted-by":"crossref","first-page":"e34688","DOI":"10.1371\/journal.pone.0034688","article-title":"The expression of microRNA and microRNA clusters in the aging heart","volume":"7","author":"Zhang","year":"2012","journal-title":"PLoS One"},{"key":"2024102814473167100_B49","doi-asserted-by":"crossref","first-page":"1401","DOI":"10.1101\/gr.193607.115","article-title":"Genome-wide annotation of microRNA primary transcript structures reveals novel regulatory mechanisms","volume":"25","author":"Chang","year":"2015","journal-title":"Genome Res."},{"key":"2024102814473167100_B50","doi-asserted-by":"crossref","first-page":"e38389","DOI":"10.7554\/eLife.38389","article-title":"Importance of miRNA stability and alternative primary miRNA isoforms in gene regulation during Drosophila development","volume":"7","author":"Zhou","year":"2018","journal-title":"eLife"},{"key":"2024102814473167100_B51","doi-asserted-by":"crossref","first-page":"840","DOI":"10.1038\/nsmb.2067","article-title":"Misregulation of miR-1 processing is associated with heart defects in myotonic dystrophy","volume":"18","author":"Rau","year":"2011","journal-title":"Nat. Struct. Mol. Biol."},{"key":"2024102814473167100_B52","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1016\/j.celrep.2013.12.025","article-title":"The Mef2 transcription network is disrupted in myotonic dystrophy heart tissue, dramatically altering miRNA and mRNA expression","volume":"6","author":"Kalsotra","year":"2014","journal-title":"Cell Rep."},{"key":"2024102814473167100_B53","doi-asserted-by":"crossref","first-page":"17587","DOI":"10.1038\/s41598-017-17816-x","article-title":"Hybrid splicing minigene and antisense oligonucleotides as efficient tools to determine functional protein\/RNA interactions","volume":"7","author":"Cywoniuk","year":"2017","journal-title":"Sci. Rep."},{"key":"2024102814473167100_B54","first-page":"10326","article-title":"Mechanistic determinants of MBNL activity","volume":"44","author":"Sznajder","year":"2016","journal-title":"Nucleic Acids Res."},{"key":"2024102814473167100_B55","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1007\/s00018-023-04927-0","article-title":"Modulatory role of RNA helicases in MBNL-dependent alternative splicing regulation","volume":"80","author":"Taylor","year":"2023","journal-title":"Cell. Mol. Life Sci."},{"key":"2024102814473167100_B56","first-page":"9711","article-title":"FUS\/TLS contributes to replication-dependent histone gene expression by interaction with U7 snRNPs and histone-specific transcription factors","volume":"43","author":"Raczynska","year":"2015","journal-title":"Nucleic. Acids. Res."},{"key":"2024102814473167100_B57","doi-asserted-by":"crossref","first-page":"e1332","DOI":"10.7717\/peerj.1332","article-title":"miRMOD: a tool for identification and analysis of 5\u2032 and 3\u2032 miRNA modifications in Next Generation sequencing small RNA data","volume":"3","author":"Kaushik","year":"2015","journal-title":"PeerJ"},{"key":"2024102814473167100_B58","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1038\/nmeth.1923","article-title":"Fast gapped-read alignment with Bowtie 2","volume":"9","author":"Langmead","year":"2012","journal-title":"Nat. Methods"},{"key":"2024102814473167100_B59","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1093\/bioinformatics\/btt656","article-title":"featureCounts: an efficient general purpose program for assigning sequence reads to genomic features","volume":"30","author":"Liao","year":"2014","journal-title":"Bioinformatics"},{"key":"2024102814473167100_B60","doi-asserted-by":"crossref","first-page":"D155","DOI":"10.1093\/nar\/gky1141","article-title":"miRBase: from microRNA sequences to function","volume":"47","author":"Kozomara","year":"2019","journal-title":"Nucleic Acids Res."},{"key":"2024102814473167100_B61","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":"2024102814473167100_B62","doi-asserted-by":"crossref","first-page":"W244","DOI":"10.1093\/nar\/gkaa467","article-title":"miRNet 2.0: network-based visual analytics for miRNA functional analysis and systems biology","volume":"48","author":"Chang","year":"2020","journal-title":"Nucleic Acids Res."},{"key":"2024102814473167100_B63","doi-asserted-by":"crossref","first-page":"1471","DOI":"10.1093\/hmg\/ddp058","article-title":"Transcriptional and post-transcriptional impact of toxic RNA in myotonic dystrophy","volume":"18","author":"Osborne","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"2024102814473167100_B64","doi-asserted-by":"crossref","first-page":"1769","DOI":"10.1126\/science.289.5485.1769","article-title":"Myotonic dystrophy in transgenic mice expressing an expanded CUG repeat","volume":"289","author":"Mankodi","year":"2000","journal-title":"Science"},{"key":"2024102814473167100_B65","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1038\/nsmb.1720","article-title":"Aberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy","volume":"17","author":"Du","year":"2010","journal-title":"Nat. Struct. Mol. Biol."},{"key":"2024102814473167100_B66","doi-asserted-by":"crossref","first-page":"dmm049016","DOI":"10.1242\/dmm.049016","article-title":"Identification of candidate miRNA biomarkers for facioscapulohumeral muscular dystrophy using DUX4-based mouse models","volume":"14","author":"Nunes","year":"2021","journal-title":"Dis Model Mech"},{"key":"2024102814473167100_B67","doi-asserted-by":"crossref","first-page":"968","DOI":"10.1038\/s41467-020-14713-2","article-title":"Pan-cancer analysis reveals cooperativity of both strands of microRNA that regulate tumorigenesis and patient survival","volume":"11","author":"Mitra","year":"2020","journal-title":"Nat. Commun."},{"key":"2024102814473167100_B68","doi-asserted-by":"crossref","first-page":"5472","DOI":"10.1073\/pnas.1907297117","article-title":"HNRNPA1-induced spliceopathy in a transgenic mouse model of myotonic dystrophy","volume":"117","author":"Li","year":"2020","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2024102814473167100_B69","doi-asserted-by":"crossref","first-page":"1812","DOI":"10.1161\/CIRCULATIONAHA.123.066860","article-title":"MBNL1 regulates programmed postnatal switching between regenerative and differentiated cardiac states","volume":"149","author":"Bailey","year":"2024","journal-title":"Circulation"},{"key":"2024102814473167100_B70","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1016\/j.molcel.2019.05.014","article-title":"3\u2032 Uridylation confers miRNAs with non-canonical target repertoires","volume":"75","author":"Yang","year":"2019","journal-title":"Mol. Cell"},{"key":"2024102814473167100_B71","doi-asserted-by":"crossref","first-page":"1881","DOI":"10.1261\/rna.2379610","article-title":"Dynamic isomiR regulation in Drosophila development","volume":"16","author":"Fernandez-Valverde","year":"2010","journal-title":"RNA"},{"key":"2024102814473167100_B72","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1016\/j.gene.2017.10.025","article-title":"IsomiR processing during differentiation of myelogenous leukemic cell line K562 by phorbol ester PMA","volume":"641","author":"Muiwo","year":"2018","journal-title":"Gene"},{"key":"2024102814473167100_B73","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":"2024102814473167100_B74","doi-asserted-by":"crossref","first-page":"168156","DOI":"10.1016\/j.jmb.2023.168156","article-title":"Alternative Splicing Outcomes Across an RNA-Binding Protein Concentration Gradient","volume":"435","author":"Ellis","year":"2023","journal-title":"J. Mol. Biol."},{"key":"2024102814473167100_B75","doi-asserted-by":"crossref","first-page":"862","DOI":"10.1002\/ana.23992","article-title":"Splicing biomarkers of disease severity in myotonic dystrophy","volume":"74","author":"Nakamori","year":"2013","journal-title":"Ann. Neurol."},{"key":"2024102814473167100_B76","first-page":"D148","article-title":"miRTarBase 2020: updates to the experimentally validated microRNA\u2013target interaction database","volume":"48","author":"Huang","year":"2019","journal-title":"Nucleic Acids Res."},{"key":"2024102814473167100_B77","doi-asserted-by":"crossref","first-page":"3939","DOI":"10.3390\/jcm9123939","article-title":"The biomarker potential of miRNAs in myotonic dystrophy type I","volume":"9","author":"Koehorst","year":"2020","journal-title":"J. Clin. Med."},{"key":"2024102814473167100_B78","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1093\/bfgp\/elz002","article-title":"Interplay between miRNAs and host genes and their role in cancer","volume":"18","author":"Liu","year":"2019","journal-title":"Brief. Funct. Genomics"},{"key":"2024102814473167100_B79","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1016\/j.molcel.2014.08.027","article-title":"Loss of MBNL leads to disruption of developmentally regulated alternative polyadenylation in RNA-mediated disease","volume":"56","author":"Batra","year":"2014","journal-title":"Mol. Cell"},{"key":"2024102814473167100_B80","doi-asserted-by":"crossref","first-page":"9424","DOI":"10.1093\/nar\/gku656","article-title":"5\u2032 isomiR variation is of functional and evolutionary importance","volume":"42","author":"Tan","year":"2014","journal-title":"Nucleic. Acids. Res."},{"key":"2024102814473167100_B81","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/s41422-018-0128-1","article-title":"A programmed wave of uridylation-primed mRNA degradation is essential for meiotic progression and mammalian spermatogenesis","volume":"29","author":"Morgan","year":"2019","journal-title":"Cell Res."},{"key":"2024102814473167100_B82","doi-asserted-by":"crossref","first-page":"882","DOI":"10.1261\/rna.060095.116","article-title":"3\u2032 Uridylation controls mature microRNA turnover during CD4 T-cell activation","volume":"23","author":"Guti\u00e9rrez-V\u00e1zquez","year":"2017","journal-title":"RNA"},{"key":"2024102814473167100_B83","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.ydbio.2015.12.013","article-title":"Muscle-specific microRNAs in skeletal muscle development","volume":"410","author":"Horak","year":"2016","journal-title":"Dev. Biol."},{"key":"2024102814473167100_B84","doi-asserted-by":"crossref","first-page":"4019","DOI":"10.3892\/mmr.2015.3275","article-title":"Role of microRNAs in skeletal muscle development and rhabdomyosarcoma (review)","volume":"11","author":"JU","year":"2015","journal-title":"Mol. Med. Rep."},{"key":"2024102814473167100_B85","doi-asserted-by":"crossref","first-page":"708278","DOI":"10.3389\/fphys.2021.708278","article-title":"Age-related exosomal and endogenous expression patterns of miR-1, miR-133a, miR-133b, and miR-206 in skeletal muscles","volume":"12","author":"Mytidou","year":"2021","journal-title":"Front. Physiol."},{"key":"2024102814473167100_B86","first-page":"3533","article-title":"Downregulation of glucose6phosphate dehydrogenase by microRNA1 inhibits the growth of pituitary tumor cells","volume":"40","author":"He","year":"2018","journal-title":"Oncol. Rep."},{"key":"2024102814473167100_B87","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1093\/cvr\/cvq264","article-title":"MicroRNA profiling during mouse ventricular maturation: a role for miR-27 modulating Mef2c expression","volume":"89","author":"Chinchilla","year":"2011","journal-title":"Cardiovasc. Res."},{"key":"2024102814473167100_B88","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1016\/j.ydbio.2011.06.039","article-title":"Pitx2c modulates Pax3+\/Pax7+ cell populations and regulates Pax3 expression by repressing miR27 expression during myogenesis","volume":"357","author":"Lozano-Velasco","year":"2011","journal-title":"Dev. Biol."},{"key":"2024102814473167100_B89","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1016\/j.omtn.2020.07.021","article-title":"Therapeutic potential of AntagomiR-23b for treating myotonic dystrophy","volume":"21","author":"Cerro-Herreros","year":"2020","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"2024102814473167100_B90","doi-asserted-by":"crossref","first-page":"2482","DOI":"10.1038\/s41467-018-04892-4","article-title":"miR-23b and miR-218 silencing increase muscleblind-like expression and alleviate myotonic dystrophy phenotypes in mammalian models","volume":"9","author":"Cerro-Herreros","year":"2018","journal-title":"Nat. Commun."},{"key":"2024102814473167100_B91","doi-asserted-by":"crossref","first-page":"1146","DOI":"10.1016\/j.omtn.2022.02.003","article-title":"Proof of concept of peptide-linked blockmiR-induced MBNL functional rescue in myotonic dystrophy type 1 mouse model","volume":"27","author":"Overby","year":"2022","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"2024102814473167100_B92","doi-asserted-by":"crossref","first-page":"e2306727120","DOI":"10.1073\/pnas.2306727120","article-title":"Parameters of clustered suboptimal miRNA biogenesis","volume":"120","author":"Shang","year":"2023","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2024102814473167100_B93","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1016\/j.molcel.2013.05.007","article-title":"Alternative splicing regulates biogenesis of miRNAs located across exon-intron junctions","volume":"50","author":"Melamed","year":"2013","journal-title":"Mol. Cell"},{"key":"2024102814473167100_B94","doi-asserted-by":"crossref","first-page":"4640","DOI":"10.1093\/nar\/gkt1413","article-title":"Interplay between pre-mRNA splicing and microRNA biogenesis within the supraspliceosome","volume":"42","author":"Agranat-Tamir","year":"2014","journal-title":"Nucleic Acids Res."},{"key":"2024102814473167100_B95","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1261\/rna.041814.113","article-title":"Biogenesis of intronic miRNAs located in clusters by independent transcription and alternative splicing","volume":"20","author":"Ramalingam","year":"2014","journal-title":"RNA"},{"key":"2024102814473167100_B96","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1007\/s42977-023-00154-7","article-title":"One locus, several functional RNAs\u2014Emerging roles of the mechanisms responsible for the sequence variability of microRNAs","volume":"74","author":"Orb\u00e1n","year":"2023","journal-title":"Biol. Futur."},{"key":"2024102814473167100_B97","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1038\/s41419-018-0769-5","article-title":"High-throughput analysis of the RNA-induced silencing complex in myotonic dystrophy type 1 patients identifies the dysregulation of miR-29c and its target ASB2","volume":"9","author":"Cappella","year":"2018","journal-title":"Cell Death. Dis."},{"key":"2024102814473167100_B98","doi-asserted-by":"crossref","first-page":"e70158","DOI":"10.1371\/journal.pone.0070158","article-title":"MicroRNA-1 downregulation increases connexin 43 displacement and induces ventricular tachyarrhythmias in rodent hypertrophic hearts","volume":"8","author":"Curcio","year":"2013","journal-title":"PLoS One"},{"key":"2024102814473167100_B99","first-page":"18","article-title":"Assessment of isomiR discrimination using commercial qPCR methods","volume":"3","author":"Magee","year":"2017","journal-title":"Noncoding RNA"},{"key":"2024102814473167100_B100","doi-asserted-by":"crossref","first-page":"11","DOI":"10.3389\/fgene.2018.00011","article-title":"Modified polyadenylation-based RT-qPCR increases selectivity of amplification of 3\u2032-MicroRNA isoforms","volume":"9","author":"Nejad","year":"2018","journal-title":"Front. Genet."},{"key":"2024102814473167100_B101","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1006\/abio.2000.4635","article-title":"Discrimination of primer 3\u2032-nucleotide mismatch by Taq DNA polymerase during polymerase chain reaction","volume":"284","author":"Ayyadevara","year":"2000","journal-title":"Anal. Biochem."},{"key":"2024102814473167100_B102","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1016\/j.jfma.2016.12.003","article-title":"Downregulation of miR-1 enhances tumorigenicity and invasiveness in oral squamous cell carcinomas","volume":"116","author":"Peng","year":"2017","journal-title":"J. Formos. Med. Assoc."},{"key":"2024102814473167100_B103","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1007\/s10072-017-2811-2","article-title":"Micro-RNA expression in muscle and fiber morphometry in myotonic dystrophy type 1","volume":"38","author":"Fritegotto","year":"2017","journal-title":"Neurolog. Sci."},{"key":"2024102814473167100_B104","doi-asserted-by":"crossref","first-page":"e56616","DOI":"10.15252\/embr.202256616","article-title":"Deregulations of miR-1 and its target Multiplexin promote dilated cardiomyopathy associated with myotonic dystrophy type 1","volume":"24","author":"Souidi","year":"2023","journal-title":"EMBO Rep."},{"key":"2024102814473167100_B105","doi-asserted-by":"crossref","first-page":"2104","DOI":"10.1242\/dev.151035","article-title":"Making muscle: skeletal myogenesis in vivo and in vitro","volume":"144","author":"Chal","year":"2017","journal-title":"Development"},{"key":"2024102814473167100_B106","doi-asserted-by":"crossref","first-page":"20844","DOI":"10.1073\/pnas.0710558105","article-title":"An intragenic MEF2-dependent enhancer directs muscle-specific expression of microRNAs 1 and 133","volume":"104","author":"Liu","year":"2007","journal-title":"Proc. Natl. Acad. Sci."},{"key":"2024102814473167100_B107","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1186\/1476-4598-10-120","article-title":"MicroRNAs in rhabdomyosarcoma: pathogenetic implications and translational potentiality","volume":"10","author":"Rota","year":"2011","journal-title":"Mol. Cancer"},{"key":"2024102814473167100_B108","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1101\/gad.1894310","article-title":"MicroRNAs coordinate an alternative splicing network during mouse postnatal heart development","volume":"24","author":"Kalsotra","year":"2010","journal-title":"Genes Dev."}],"container-title":["Nucleic Acids Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/nar\/article-pdf\/52\/19\/12055\/60142282\/gkae774.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/nar\/article-pdf\/52\/19\/12055\/60142282\/gkae774.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,28]],"date-time":"2024-10-28T14:49:37Z","timestamp":1730126977000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/nar\/article\/52\/19\/12055\/7754984"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,11]]},"references-count":108,"journal-issue":{"issue":"19","published-online":{"date-parts":[[2024,9,11]]},"published-print":{"date-parts":[[2024,10,28]]}},"URL":"https:\/\/doi.org\/10.1093\/nar\/gkae774","relation":{},"ISSN":["0305-1048","1362-4962"],"issn-type":[{"value":"0305-1048","type":"print"},{"value":"1362-4962","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2024,10,28]]},"published":{"date-parts":[[2024,9,11]]}}}