{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T18:10:58Z","timestamp":1790878258673,"version":"4.1.0"},"reference-count":74,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2025,11,6]],"date-time":"2025-11-06T00:00:00Z","timestamp":1762387200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100016047","name":"Science Fund of the Republic of Serbia","doi-asserted-by":"crossref","award":["7754217"],"award-info":[{"award-number":["7754217"]}],"id":[{"id":"10.13039\/501100016047","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Myotonic dystrophy type 1 (DM1) is a progressive multisystemic disease caused by a CTG repeat expansion in the DMPK gene. The toxic mutant mRNA sequesters MBNL proteins, disrupting global RNA metabolism. Although alternative splicing in DM1 skeletal muscle pathology has been extensively studied, early-stage transcriptomic changes remained uncharacterized. To gain deeper and contextual insight into DM1 transcriptome, we performed the first Weighted Gene Co-expression Network Analysis (WGCNA) on skeletal muscle RNA sequencing data from the widely used DM1 mouse model HSALR (~250 CTG repeats). We identified 532 core genes using data from 16-week-old mice, an age before the onset of muscle weakness. Additional differential expression analysis across multiple HSALR datasets revealed 42 common up-regulated coding and non-coding genes. Within identified core genes, the pathway gene-pair signature analysis enabled contextual selection of functionally related genes involved in maintaining proteostasis, including endoplasmic reticulum (ER) protein processing, the ubiquitin-proteasome system (UPS), macroautophagy and mitophagy, and muscle contraction. The enrichment of ER protein processing with prevailing core genes related to ER-associated degradation suggests adaptive chaperone and UPS activation, while core genes such as Ambra1, Mfn2, and Usp30 indicate adaptations in mitochondrial quality control. Coordinated early alterations in processes maintaining protein homeostasis, critical for muscle mass and function, possibly reflect a response to cellular stress due to repeat expansion and appears before muscle weakness development. Although the study relies exclusively on transcriptomic analyses, it offers a comprehensive, hypothesis-generating perspective that pinpoints candidate pathways, preceding muscle weakness, for future mechanistic validation.<\/jats:p>","DOI":"10.3390\/ijms262110793","type":"journal-article","created":{"date-parts":[[2025,11,6]],"date-time":"2025-11-06T17:51:43Z","timestamp":1762451503000},"page":"10793","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["HSALR Mice Exhibit Co-Expression of Proteostasis Genes Prior to Development of Muscle Weakness"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-0350-3246","authenticated-orcid":false,"given":"Dusan M.","family":"Lazic","sequence":"first","affiliation":[{"name":"Center for Human Molecular Genetics, Faculty of Biology, University of Belgrade, Studentski Trg 16, 11000 Belgrade, Serbia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8056-5065","authenticated-orcid":false,"given":"Vladimir M.","family":"Jovanovic","sequence":"additional","affiliation":[{"name":"Department of Biology, Chemistry and Pharmacy, Freie Universit\u00e4t Berlin, K\u00f6nigin-Luise-Str. 1-3, 14195 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6291-5527","authenticated-orcid":false,"given":"Jelena","family":"Karanovic","sequence":"additional","affiliation":[{"name":"Gene Regulation in Cancer Group, Institute of Molecular Genetics and Genetic Engineering, University of Belgrade, Vojvode Stepe 444A, 11042 Belgrade, Serbia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2079-4077","authenticated-orcid":false,"given":"Dusanka","family":"Savic-Pavicevic","sequence":"additional","affiliation":[{"name":"Center for Human Molecular Genetics, Faculty of Biology, University of Belgrade, Studentski Trg 16, 11000 Belgrade, Serbia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8613-797X","authenticated-orcid":false,"given":"Bogdan","family":"Jovanovic","sequence":"additional","affiliation":[{"name":"Center for Human Molecular Genetics, Faculty of Biology, University of Belgrade, Studentski Trg 16, 11000 Belgrade, Serbia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,11,6]]},"reference":[{"key":"ref_1","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":"ref_2","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1016\/j.neurol.2016.08.003","article-title":"Unravelling the Myotonic Dystrophy Type 1 Clinical Spectrum: A Systematic Registry-Based Study with Implications for Disease Classification","volume":"172","author":"Dogan","year":"2016","journal-title":"Rev. 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