{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T15:27:47Z","timestamp":1790695667738,"version":"4.1.0"},"reference-count":100,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2021,1,26]],"date-time":"2021-01-26T00:00:00Z","timestamp":1611619200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003141","name":"Consejo Nacional de Ciencia y Tecnolog\u00eda","doi-asserted-by":"publisher","award":["CB-2012-01\/183697, CB258043 and CB258268"],"award-info":[{"award-number":["CB-2012-01\/183697, CB258043 and CB258268"]}],"id":[{"id":"10.13039\/501100003141","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomolecules"],"abstract":"<jats:p>Myotonic dystrophy type 1 (DM1), the most frequent inherited muscular dystrophy in adults, is caused by the CTG repeat expansion in the 3\u2032UTR of the DMPK gene. Mutant DMPK RNA accumulates in nuclear foci altering diverse cellular functions including alternative splicing regulation. DM1 is a multisystemic condition, with debilitating central nervous system alterations. Although a defective neuroglia communication has been described as a contributor of the brain pathology in DM1, the specific cellular and molecular events potentially affected in glia cells have not been totally recognized. Thus, to study the effects of DM1 mutation on glial physiology, in this work, we have established an inducible DM1 model derived from the MIO-M1 cell line expressing 648 CUG repeats. This new model recreated the molecular hallmarks of DM1 elicited by a toxic RNA gain-of-function mechanism: accumulation of RNA foci colocalized with MBNL proteins and dysregulation of alternative splicing. By applying a microarray whole-transcriptome approach, we identified several gene changes associated with DM1 mutation in MIO-M1 cells, including the immune mediators CXCL10, CCL5, CXCL8, TNFAIP3, and TNFRSF9, as well as the microRNAs miR-222, miR-448, among others, as potential regulators. A gene ontology enrichment analyses revealed that inflammation and immune response emerged as major cellular deregulated processes in the MIO-M1 DM1 cells. Our findings indicate the involvement of an altered immune response in glia cells, opening new windows for the study of glia as potential contributor of the CNS symptoms in DM1.<\/jats:p>","DOI":"10.3390\/biom11020159","type":"journal-article","created":{"date-parts":[[2021,1,26]],"date-time":"2021-01-26T08:29:16Z","timestamp":1611649756000},"page":"159","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Transcriptome Analysis Reveals Altered Inflammatory Pathway in an Inducible Glial Cell Model of Myotonic Dystrophy Type 1"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3478-4427","authenticated-orcid":false,"given":"Cuauhtli N.","family":"Azotla-Vilchis","sequence":"first","affiliation":[{"name":"Laboratory of Genomic Medicine, Department of Genetics, Instituto Nacional de Rehabilitaci\u00f3n, Luis Guillermo Ibarra Ibarra, Mexico City 14389, Mexico"},{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados, CINVESTAV-IPN, Mexico City 07360, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Sanchez-Celis","sequence":"additional","affiliation":[{"name":"Laboratory of Genomic Medicine, Department of Genetics, Instituto Nacional de Rehabilitaci\u00f3n, Luis Guillermo Ibarra Ibarra, Mexico City 14389, Mexico"},{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados, CINVESTAV-IPN, Mexico City 07360, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis E.","family":"Agonizantes-Ju\u00e1rez","sequence":"additional","affiliation":[{"name":"Laboratory of Genomic Medicine, Department of Genetics, Instituto Nacional de Rehabilitaci\u00f3n, Luis Guillermo Ibarra Ibarra, Mexico City 14389, Mexico"},{"name":"Escuela Nacional de Ciencias Biologicas-Instituto Polit\u00e9cnico Nacional, Mexico City 11340, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5269-7124","authenticated-orcid":false,"given":"Roc\u00edo","family":"Su\u00e1rez-S\u00e1nchez","sequence":"additional","affiliation":[{"name":"Laboratory of Genomic Medicine, Department of Genetics, Instituto Nacional de Rehabilitaci\u00f3n, Luis Guillermo Ibarra Ibarra, Mexico City 14389, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7624-9986","authenticated-orcid":false,"given":"J. Manuel","family":"Hern\u00e1ndez-Hern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados, CINVESTAV-IPN, Mexico City 07360, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge","family":"Pe\u00f1a","sequence":"additional","affiliation":[{"name":"Computational and Integrative Genomics Laboratory, Instituto Nacional de Medicina Gen\u00f3mica, Mexico City 14610, Mexico"},{"name":"Institute of Mathematical Sciences, Claremont Graduate University, Claremont, CA 91711, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karla","family":"V\u00e1zquez-Santill\u00e1n","sequence":"additional","affiliation":[{"name":"Epigenetics Laboratory, Instituto Nacional de Medicina Genomica, Mexico City 14610, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norberto","family":"Leyva-Garc\u00eda","sequence":"additional","affiliation":[{"name":"Laboratory of Genomic Medicine, Department of Genetics, Instituto Nacional de Rehabilitaci\u00f3n, Luis Guillermo Ibarra Ibarra, Mexico City 14389, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9594-8114","authenticated-orcid":false,"given":"Arturo","family":"Ortega","sequence":"additional","affiliation":[{"name":"Department of Toxicology, Centro de Investigaci\u00f3n y de Estudios Avanzados, CINVESTAV-IPN, Mexico City 07360, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vilma","family":"Maldonado","sequence":"additional","affiliation":[{"name":"Epigenetics Laboratory, Instituto Nacional de Medicina Genomica, Mexico City 14610, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Rangel","sequence":"additional","affiliation":[{"name":"Computational and Integrative Genomics Laboratory, Instituto Nacional de Medicina Gen\u00f3mica, Mexico City 14610, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4399-4618","authenticated-orcid":false,"given":"Jonathan J.","family":"Maga\u00f1a","sequence":"additional","affiliation":[{"name":"Laboratory of Genomic Medicine, Department of Genetics, Instituto Nacional de Rehabilitaci\u00f3n, Luis Guillermo Ibarra Ibarra, Mexico City 14389, Mexico"},{"name":"School of Engineering and Sciences, Department of Bioengineering, Tecnol\u00f3gico de Monterrey-Campus, Mexico City 14380, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bulmaro","family":"Cisneros","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados, CINVESTAV-IPN, Mexico City 07360, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6388-2459","authenticated-orcid":false,"given":"Oscar","family":"Hern\u00e1ndez-Hern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Laboratory of Genomic Medicine, Department of Genetics, Instituto Nacional de Rehabilitaci\u00f3n, Luis Guillermo Ibarra Ibarra, Mexico City 14389, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,1,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1159\/000369343","article-title":"Prevalence of muscular dystrophies: A systematic literature review","volume":"43","author":"Theadom","year":"2014","journal-title":"Neuroepidemiology"},{"key":"ref_2","first-page":"385","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":"69","author":"Brook","year":"1992","journal-title":"Cell"},{"key":"ref_3","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":"ref_4","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":"ref_5","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1016\/S1474-4422(12)70204-1","article-title":"The myotonic dystrophies: Molecular, clinical, and therapeutic challenges","volume":"11","author":"Udd","year":"2012","journal-title":"Lancet Neurol."},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Dogan, C., De Antonio, M., Hamroun, D., Varet, H., Fabbro, M., Rougier, F., Amarof, K., Arne Bes, M.C., Bedat-Millet, A.L., and Behin, A. (2016). Gender as a Modifying Factor Influencing Myotonic Dystrophy Type 1 Phenotype Severity and Mortality: A Nationwide Multiple Databases Cross-Sectional Observational Study. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0148264"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"101","DOI":"10.3389\/fncel.2017.00101","article-title":"Myotonic Dystrophies: State of the Art of New Therapeutic Developments for the CNS","volume":"11","author":"Gourdon","year":"2017","journal-title":"Front. Cell Neurosci."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1016\/j.nmd.2011.04.009","article-title":"A new window on neurocognitive dysfunction in the childhood form of myotonic dystrophy type 1 (DM1)","volume":"21","author":"Angeard","year":"2011","journal-title":"Neuromuscul. Disord."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1111\/j.1469-8749.2012.04379.x","article-title":"Psychiatric and cognitive phenotype of childhood myotonic dystrophy type 1","volume":"54","author":"Douniol","year":"2012","journal-title":"Dev. Med. Child Neurol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1111\/ane.12356","article-title":"Psychological characteristics of patients with myotonic dystrophy type 1","volume":"132","author":"Bertrand","year":"2015","journal-title":"Acta Neurol. Scand."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"751","DOI":"10.3389\/fneur.2018.00751","article-title":"Neuropsychological and Psychological Functioning Aspects in Myotonic Dystrophy Type 1 Patients in Italy","volume":"9","author":"Callus","year":"2018","journal-title":"Front. Neurol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.clineuro.2014.08.021","article-title":"Significant impact of behavioral and cognitive impairment on quality of life in patients with myotonic dystrophy type 1","volume":"126","author":"Peric","year":"2014","journal-title":"Clin. Neurol. Neurosurg."},{"key":"ref_13","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":"ref_14","doi-asserted-by":"crossref","first-page":"3079","DOI":"10.1093\/hmg\/ddh327","article-title":"Myotonic dystrophy type 1 is associated with nuclear foci of mutant RNA, sequestration of muscleblind proteins and deregulated alternative splicing in neurons","volume":"13","author":"Jiang","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"ref_15","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":"ref_16","doi-asserted-by":"crossref","first-page":"519","DOI":"10.3389\/fneur.2018.00519","article-title":"Of Mice and Men: Advances in the Understanding of Neuromuscular Aspects of Myotonic Dystrophy","volume":"9","author":"Braz","year":"2018","journal-title":"Front. Neurol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1016\/j.bbadis.2014.05.019","article-title":"Myotonic dystrophies: An update on clinical aspects, genetic, pathology, and molecular pathomechanisms","volume":"1852","author":"Meola","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_18","unstructured":"Rosenberg, R.N., and Pascual, J.M. (2020). The myotonic dystrophies. Rosenerg\u2019s Molecular and Genetic Basis of Neurological and Psychiatric Disease, Academic Press. [6th ed.]."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"R116","DOI":"10.1093\/hmg\/ddr343","article-title":"Myotonic dystrophy, when simple repeats reveal complex pathogenic entities: New findings and future challenges","volume":"20","author":"Sicot","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Sznajder, L.J., and Swanson, M.S. (2019). Short Tandem Repeat Expansions and RNA-Mediated Pathogenesis in Myotonic Dystrophy. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20133365"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1016\/j.molcel.2005.09.002","article-title":"Antisense transcription and heterochromatin at the DM1 CTG repeats are constrained by CTCF","volume":"20","author":"Cho","year":"2005","journal-title":"Mol. Cell"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1146\/annurev-neuro-070918-050405","article-title":"Repeat-Associated Non-ATG Translation: Molecular Mechanisms and Contribution to Neurological Disease","volume":"42","author":"Nguyen","year":"2019","journal-title":"Annu. Rev. Neurosci."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1073\/pnas.1013343108","article-title":"Non-ATG-initiated translation directed by microsatellite expansions","volume":"108","author":"Zu","year":"2011","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_24","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":"ref_25","doi-asserted-by":"crossref","first-page":"7820","DOI":"10.1074\/jbc.M005960200","article-title":"RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1","volume":"276","author":"Timchenko","year":"2001","journal-title":"J. Biol. Chem."},{"key":"ref_26","first-page":"957","article-title":"Myotonic dystrophy CTG expansion affects synaptic vesicle proteins, neurotransmission and mouse behaviour","volume":"136 (Pt 3)","author":"Sicot","year":"2013","journal-title":"Brain"},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Suenaga, K., Lee, K.Y., Nakamori, M., Tatsumi, Y., Takahashi, M.P., Fujimura, H., Jinnai, K., Yoshikawa, H., Du, H., and Ares, M. (2012). Muscleblind-like 1 knockout mice reveal novel splicing defects in the myotonic dystrophy brain. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0033218"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1126\/science.aat0473","article-title":"Glia as architects of central nervous system formation and function","volume":"362","author":"Allen","year":"2018","journal-title":"Science"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1002\/mus.21352","article-title":"DMPK protein isoforms are differentially expressed in myogenic and neural cell lineages","volume":"40","author":"Mulders","year":"2009","journal-title":"Muscle Nerve"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"2718","DOI":"10.1016\/j.celrep.2017.06.006","article-title":"Downregulation of the Glial GLT1 Glutamate Transporter and Purkinje Cell Dysfunction in a Mouse Model of Myotonic Dystrophy","volume":"19","author":"Sicot","year":"2017","journal-title":"Cell Rep."},{"key":"ref_31","first-page":"864","article-title":"In vitro characterization of a spontaneously immortalized human Muller cell line (MIO-M1)","volume":"43","author":"Limb","year":"2002","journal-title":"Investig. Ophthalmol. Vis. Sci."},{"key":"ref_32","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 (Pt 13)","author":"Ho","year":"2005","journal-title":"J. Cell Sci."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1106","DOI":"10.1016\/j.nmd.2017.09.004","article-title":"Origin of the myotonic dystrophy type 1 mutation in Mexican population and influence of Amerindian ancestry on CTG repeat allelic distribution","volume":"27","author":"Gomez","year":"2017","journal-title":"Neuromuscul. Disord."},{"key":"ref_34","doi-asserted-by":"crossref","unstructured":"Tome, S., Nicole, A., Gomes-Pereira, M., and Gourdon, G. (2014). Non-radioactive detection of trinucleotide repeat size variability. PLoS Curr., 6.","DOI":"10.1371\/currents.md.ad50113b899fa1352ce70c087eead706"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e15","DOI":"10.1093\/nar\/gng015","article-title":"Summaries of Affymetrix GeneChip probe level data","volume":"31","author":"Irizarry","year":"2003","journal-title":"Nucleic Acids Res."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1093\/bioinformatics\/19.2.185","article-title":"A comparison of normalization methods for high density oligonucleotide array data based on variance and bias","volume":"19","author":"Bolstad","year":"2003","journal-title":"Bioinformatics"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"e47","DOI":"10.1093\/nar\/gkv007","article-title":"Limma powers differential expression analyses for RNA-sequencing and microarray studies","volume":"43","author":"Ritchie","year":"2015","journal-title":"Nucleic Acids Res."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"Article3","DOI":"10.2202\/1544-6115.1027","article-title":"Linear models and empirical bayes methods for assessing differential expression in microarray experiments","volume":"3","author":"Smyth","year":"2004","journal-title":"Stat. Appl. Genet. Mol. Biol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"15545","DOI":"10.1073\/pnas.0506580102","article-title":"Gene set enrichment analysis: A knowledge-based approach for interpreting genome-wide expression profiles","volume":"102","author":"Subramanian","year":"2005","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"D360","DOI":"10.1093\/nar\/gkx1144","article-title":"mirDIP 4.1-integrative database of human microRNA target predictions","volume":"46","author":"Tokar","year":"2018","journal-title":"Nucleic Acids Res."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"D127","DOI":"10.1093\/nar\/gkz757","article-title":"miRDB: An online database for prediction of functional microRNA targets","volume":"48","author":"Chen","year":"2020","journal-title":"Nucleic Acids Res."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"2628","DOI":"10.1093\/bioinformatics\/btz931","article-title":"ShinyGO: A graphical gene-set enrichment tool for animals and plants","volume":"36","author":"Ge","year":"2020","journal-title":"Bioinformatics"},{"key":"ref_43","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":"ref_44","doi-asserted-by":"crossref","first-page":"e25553","DOI":"10.4161\/rdis.25553","article-title":"Synaptic protein dysregulation in myotonic dystrophy type 1, Disease neuropathogenesis beyond missplicing","volume":"1","author":"Sicot","year":"2013","journal-title":"Rare Dis."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"2247","DOI":"10.1093\/hmg\/ddx115","article-title":"Reduced cytoplasmic MBNL1 is an early event in a brain-specific mouse model of myotonic dystrophy","volume":"26","author":"Wang","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"ref_46","first-page":"cm1","article-title":"Interleukin-1 (IL-1) pathway","volume":"3","author":"Weber","year":"2010","journal-title":"Sci. Signal."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"ra66","DOI":"10.1126\/scisignal.2000387","article-title":"Two mechanistically and temporally distinct NF-kappaB activation pathways in IL-1 signaling","volume":"2","author":"Yamazaki","year":"2009","journal-title":"Sci. Signal."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1007\/s12640-020-00263-4","article-title":"Fluoride Exposure Affects Glutamine Uptake in Muller Glia Cells","volume":"38","author":"Ortega","year":"2020","journal-title":"Neurotox Res."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"2033","DOI":"10.1634\/stemcells.2006-0724","article-title":"MIO-M1 cells and similar muller glial cell lines derived from adult human retina exhibit neural stem cell characteristics","volume":"25","author":"Lawrence","year":"2007","journal-title":"Stem Cells"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1312","DOI":"10.1093\/hmg\/ddy432","article-title":"Transcriptome alterations in myotonic dystrophy skeletal muscle and heart","volume":"28","author":"Wang","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1101\/gr.184390.114","article-title":"Antagonistic regulation of mRNA expression and splicing by CELF and MBNL proteins","volume":"25","author":"Wang","year":"2015","journal-title":"Genome Res."},{"key":"ref_52","doi-asserted-by":"crossref","unstructured":"Lopez Castel, A., Overby, S.J., and Artero, R. (2019). MicroRNA-Based Therapeutic Perspectives in Myotonic Dystrophy. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20225600"},{"key":"ref_53","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":"ref_54","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":"ref_55","first-page":"143","article-title":"Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1","volume":"7","author":"Todd","year":"2014","journal-title":"Dis. Models Mech."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.2337\/diabetes.48.5.1108","article-title":"Tumor necrosis factor system activity is associated with insulin resistance and dyslipidemia in myotonic dystrophy","volume":"48","author":"Molina","year":"1999","journal-title":"Diabetes"},{"key":"ref_57","first-page":"3169","article-title":"Abnormal cytokine and adrenocortical hormone regulation in myotonic dystrophy","volume":"85","author":"Johansson","year":"2000","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/S0022-510X(02)00193-4","article-title":"Tumor necrosis factor-alpha and myocardial function in patients with myotonic dystrophy type 1","volume":"201","author":"Mammarella","year":"2002","journal-title":"J. Neurol. Sci."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"S58","DOI":"10.1212\/01.wnl.0000275234.43506.9b","article-title":"Dual role of inflammation in CNS disease","volume":"68","author":"Hohlfeld","year":"2007","journal-title":"Neurology"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1016\/j.immuni.2016.04.005","article-title":"Brain Endothelial- and Epithelial-Specific Interferon Receptor Chain 1 Drives Virus-Induced Sickness Behavior and Cognitive Impairment","volume":"44","author":"Blank","year":"2016","journal-title":"Immunity"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"15862","DOI":"10.1038\/srep15862","article-title":"The Chemokine MIP-1alpha\/CCL3 impairs mouse hippocampal synaptic transmission, plasticity and memory","volume":"5","author":"Marciniak","year":"2015","journal-title":"Sci. Rep."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1677\/JME-06-0035","article-title":"Chemokines and chemokine receptors in the brain: Implication in neuroendocrine regulation","volume":"38","author":"Callewaere","year":"2007","journal-title":"J. Mol. Endocrinol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1176\/appi.ajp.161.5.889","article-title":"Elevated maternal interleukin-8 levels and risk of schizophrenia in adult offspring","volume":"161","author":"Brown","year":"2004","journal-title":"Am. J. Psychiatry"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1038\/mp.2017.205","article-title":"Chemokines in depression in health and in inflammatory illness: A systematic review and meta-analysis","volume":"23","author":"Leighton","year":"2018","journal-title":"Mol. Psychiatry"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1016\/j.schres.2010.05.014","article-title":"Structural brain alterations in schizophrenia following fetal exposure to the inflammatory cytokine interleukin-8","volume":"121","author":"Ellman","year":"2010","journal-title":"Schizophr. Res."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.3389\/fimmu.2017.01079","article-title":"CCL5-Glutamate Cross-Talk in Astrocyte-Neuron Communication in Multiple Sclerosis","volume":"8","author":"Pittaluga","year":"2017","journal-title":"Front. Immunol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"357","DOI":"10.3389\/fncel.2015.00357","article-title":"Systematic Review of the Neurobiological Relevance of Chemokines to Psychiatric Disorders","volume":"9","author":"Stuart","year":"2015","journal-title":"Front. Cell Neurosci."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1016\/S0165-5728(96)00154-3","article-title":"Induction of RANTES expression by astrocytes and astrocytoma cell lines","volume":"71","author":"Barnes","year":"1996","journal-title":"J. Neuroimmunol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/S0165-6147(99)01308-5","article-title":"Chemokines and chemokine receptors in the CNS: A possible role in neuroinflammation and patterning","volume":"20","author":"Mennicken","year":"1999","journal-title":"Trends Pharm. Sci."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1002\/jnr.10727","article-title":"Expression of CD137 and its ligand in human neurons, astrocytes, and microglia: Modulation by FGF-2","volume":"74","author":"Reali","year":"2003","journal-title":"J. NeuroSci. Res."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1186\/1742-2094-9-173","article-title":"CD137 ligand activated microglia induces oligodendrocyte apoptosis via reactive oxygen species","volume":"9","author":"Yeo","year":"2012","journal-title":"J. Neuroinflamm."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"2036","DOI":"10.1038\/s41467-018-04376-5","article-title":"A20 critically controls microglia activation and inhibits inflammasome-dependent neuroinflammation","volume":"9","author":"Voet","year":"2018","journal-title":"Nat. Commun."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"2978","DOI":"10.1093\/cercor\/bhy164","article-title":"Methylphenidate Attenuates the Cognitive and Mood Alterations Observed in Mbnl2 Knockout Mice and Reduces Microglia Overexpression","volume":"29","author":"Gener","year":"2019","journal-title":"Cereb. Cortex"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1490","DOI":"10.1016\/j.bbadis.2015.04.010","article-title":"Celf1 regulates cell cycle and is partially responsible for defective myoblast differentiation in myotonic dystrophy RNA toxicity","volume":"1852","author":"Peng","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1016\/j.bbamcr.2005.05.009","article-title":"Signaling via NF-kappaB in the nervous system","volume":"1745","author":"Kaltschmidt","year":"2005","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1042\/BST20130156","article-title":"NF-kappaB and the cell cycle","volume":"42","author":"Ledoux","year":"2014","journal-title":"Biochem. Soc. Trans."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"725","DOI":"10.1038\/nri910","article-title":"NF-kappaB regulation in the immune system","volume":"2","author":"Li","year":"2002","journal-title":"Nat. Rev. Immunol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1007\/s11064-005-6961-x","article-title":"NF-kappaB in the survival and plasticity of neurons","volume":"30","author":"Mattson","year":"2005","journal-title":"Neurochem. Res."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1016\/0092-8674(86)90807-X","article-title":"Inducibility of kappa immunoglobulin enhancer-binding protein Nf-kappa B by a posttranslational mechanism","volume":"47","author":"Sen","year":"1986","journal-title":"Cell"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1038\/cmi.2014.35","article-title":"K63-linked polyubiquitination of IRF1, An essential step in the IL-1 signaling cascade","volume":"11","author":"Wang","year":"2014","journal-title":"Cell Mol. Immunol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"734","DOI":"10.1124\/jpet.106.118125","article-title":"Repression of inflammatory gene expression in human pulmonary epithelial cells by small-molecule IkappaB kinase inhibitors","volume":"321","author":"Newton","year":"2007","journal-title":"J. Pharm. Exp."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"3863","DOI":"10.1074\/jbc.M110572200","article-title":"Distinct roles of the Ikappa B kinase alpha and beta subunits in liberating nuclear factor kappa B (NF-kappa B) from Ikappa B and in phosphorylating the p65 subunit of NF-kappa B","volume":"277","author":"Sizemore","year":"2002","journal-title":"J. Biol. Chem."},{"key":"ref_83","doi-asserted-by":"crossref","unstructured":"Koehorst, E., Ballester-Lopez, A., Arechavala-Gomeza, V., Martinez-Pineiro, A., and Nogales-Gadea, G. (2020). The Biomarker Potential of miRNAs in Myotonic Dystrophy Type, I. J. Clin. Med., 9.","DOI":"10.3390\/jcm9123939"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"3737","DOI":"10.3892\/mmr.2017.7059","article-title":"Investigation of the Mol.ecular mechanisms underlying myotonic dystrophy types 1 and 2 cataracts using microRNAtarget gene networks","volume":"16","author":"Shao","year":"2017","journal-title":"Mol. Med. Rep."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1038\/s41419-020-03112-6","article-title":"miR-322\/-503 rescues myoblast defects in myotonic dystrophy type 1 cell model by targeting CUG repeats","volume":"11","author":"Shen","year":"2020","journal-title":"Cell Death Dis."},{"key":"ref_86","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":"ref_87","doi-asserted-by":"crossref","first-page":"21179","DOI":"10.1073\/pnas.0909167106","article-title":"MicroRNAs couple cell fate and developmental timing in retina","volume":"106","author":"Decembrini","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"R173","DOI":"10.1186\/gb-2007-8-8-r173","article-title":"MicroRNAs show a wide diversity of expression profiles in the developing and mature central nervous system","volume":"8","author":"Kapsimali","year":"2007","journal-title":"Genome Biol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1186\/s12935-019-0821-2","article-title":"Inhibition of microRNA-103a inhibits the activation of astrocytes in hippocampus tissues and improves the pathological injury of neurons of epilepsy rats by regulating BDNF","volume":"19","author":"Zheng","year":"2019","journal-title":"Cancer Cell Int."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/j.archger.2019.01.007","article-title":"Associations of serum microRNA-20a, -27a, and -103a with cognitive function in a Japanese population: The Yakumo study","volume":"82","author":"Kondo","year":"2019","journal-title":"Arch. Gerontol. Geriatr."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"1971","DOI":"10.1074\/jbc.M807530200","article-title":"MicroRNA-298 and microRNA-328 regulate expression of mouse beta-amyloid precursor protein-converting enzyme 1","volume":"284","author":"Boissonneault","year":"2009","journal-title":"J. Biol. Chem."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1097\/WNR.0b013e3280148e8b","article-title":"Micro-RNA speciation in fetal, adult and Alzheimer\u2019s disease hippocampus","volume":"18","author":"Lukiw","year":"2007","journal-title":"Neuroreport"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"7219","DOI":"10.1093\/nar\/gkq575","article-title":"A myriad of miRNA variants in control and Huntington\u2019s disease brain regions detected by massively parallel sequencing","volume":"38","author":"Marti","year":"2010","journal-title":"Nucleic Acids Res."},{"key":"ref_94","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\u2019s disease","volume":"28","author":"Packer","year":"2008","journal-title":"J. Neurosci."},{"key":"ref_95","first-page":"189","article-title":"Natural compounds that modulate BACE1-processing of amyloid-beta precursor protein in Alzheimer\u2019s disease","volume":"14","author":"Zhang","year":"2012","journal-title":"Discov. Med."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1016\/j.yexcr.2018.05.014","article-title":"Exosome-mediated miR-222 transferring: An insight into NF-kappaB-mediated breast cancer metastasis","volume":"369","author":"Ding","year":"2018","journal-title":"Exp. Cell Res."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"3892","DOI":"10.1093\/nar\/gkr006","article-title":"NF-kB and c-Jun induce the expression of the oncogenic miR-221 and miR-222 in prostate carcinoma and glioblastoma cells","volume":"39","author":"Galardi","year":"2011","journal-title":"Nucleic Acids Res."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1038\/cdd.2010.103","article-title":"Involvement of NF-kappaB\/miR-448 regulatory feedback loop in chemotherapy-induced epithelial-mesenchymal transition of breast cancer cells","volume":"18","author":"Li","year":"2011","journal-title":"Cell Death Differ."},{"key":"ref_99","first-page":"351","article-title":"MicroRNA protocols. Perspectives","volume":"342","author":"Ying","year":"2006","journal-title":"Methods Mol. Biol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1038\/nrg3162","article-title":"MicroRNAs and their targets: Recognition, regulation and an emerging reciprocal relationship","volume":"13","author":"Pasquinelli","year":"2012","journal-title":"Nat. Rev. Genet."}],"container-title":["Biomolecules"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2218-273X\/11\/2\/159\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T05:15:31Z","timestamp":1760159731000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2218-273X\/11\/2\/159"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,26]]},"references-count":100,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2021,2]]}},"alternative-id":["biom11020159"],"URL":"https:\/\/doi.org\/10.3390\/biom11020159","relation":{},"ISSN":["2218-273X"],"issn-type":[{"value":"2218-273X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,1,26]]}}}