{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T06:19:22Z","timestamp":1783491562261,"version":"3.55.0"},"reference-count":140,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T00:00:00Z","timestamp":1783468800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002613","name":"Ulsan National Institute of Science and Technology","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002613","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Myopathies are a heterogeneous group of skeletal muscle disorders caused by genetic mutations or acquired insults, including inflammation, infection, endocrine imbalance, and toxic exposure. Myopathies affect a substantial number of individuals worldwide and are a significant cause of chronic muscle weakness and disability. Despite diverse etiologies, progressive myofiber injury and degeneration underlie the functional decline across disease subtypes. Accumulating evidence indicates that innate immune activation and regulated myofiber death pathways, including apoptosis, necroptosis, and pyroptosis, contribute to disease progression in selected genetic and acquired myopathies and may represent increasingly actionable therapeutic targets. This review focuses specifically on the interplay between innate immune signaling and the regulation of cell death pathways in skeletal muscle across diverse myopathies. We discuss pattern recognition receptors, inflammasome activation, and cytokine-driven pathways, such as tumor necrosis factor-alpha (TNF-\u03b1), type I interferons (IFNs), and interleukin (IL) family signaling, highlighting how these mechanisms amplify inflammation, impair regeneration, and promote myofiber degeneration. To illustrate category-specific mechanisms, we selected representative disorders from each major myopathy group, including Duchenne muscular dystrophy (DMD) as a prototypical DAMP-driven muscular dystrophy, myotonic dystrophy type 1 (DM1) as a model of secondary innate immune activation associated with RNA toxicity-induced cellular stress, dermatomyositis (DM) as a representative inflammatory myopathy, and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated myopathy as a clinically relevant model of virus-related muscle involvement and systemic inflammation-associated muscle injury. By integrating evidence across these disease contexts, this review highlights convergent mechanisms in which innate immune dysregulation and regulated myofiber death drive muscle pathology and provide rational targets for mechanism-based therapeutic strategies.<\/jats:p>","DOI":"10.3389\/fimmu.2026.1871966","type":"journal-article","created":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T05:59:21Z","timestamp":1783490361000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Convergent innate immune and regulated cell-death pathways in selected myopathies"],"prefix":"10.3389","volume":"17","author":[{"given":"Moe","family":"Yamashita","sequence":"first","affiliation":[{"name":"Department of Biological Sciences, Ulsan National Institute of Science and Technology (UNIST)","place":["Ulsan, Republic of Korea"]},{"name":"Department of Biotechnology and Life Science, Tokyo University of Agriculture and Technology","place":["Tokyo, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaewoo","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Biological Sciences, Ulsan National Institute of Science and Technology (UNIST)","place":["Ulsan, Republic of Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sehee","family":"Park","sequence":"additional","affiliation":[{"name":"Division of Acute Viral Disease, Center for Emerging Virus Research, National Institute of Infectious Diseases, National Institute of Health","place":["Cheongju, Republic of Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hae Ji","family":"Kang","sequence":"additional","affiliation":[{"name":"Division of Acute Viral Disease, Center for Emerging Virus Research, National Institute of Infectious Diseases, National Institute of Health","place":["Cheongju, Republic of Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoon-Seok","family":"Chung","sequence":"additional","affiliation":[{"name":"Division of Acute Viral Disease, Center for Emerging Virus Research, National Institute of Infectious Diseases, National Institute of Health","place":["Cheongju, Republic of Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seon Ah","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Life Science, Ewha Womans University","place":["Seoul, Republic of Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"SangJoon","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Biological Sciences, Ulsan National Institute of Science and Technology (UNIST)","place":["Ulsan, Republic of Korea"]},{"name":"Graduate School of Health Science and Technology, Ulsan National Institute of Science and Technology (UNIST)","place":["Ulsan, Republic of Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,7,8]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(02)07815-7","article-title":"The muscular dystrophies","volume":"359","author":"Emery","year":"2002","journal-title":"Lancet"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199111213252107","article-title":"Polymyositis, dermatomyositis, and inclusion-body myositis","volume":"325","author":"Dalakas","year":"1991","journal-title":"N Engl J Med"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(03)14368-1","article-title":"Polymyositis and dermatomyositis","volume":"362","author":"Dalakas","year":"2003","journal-title":"Lancet"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197502132920706","article-title":"Polymyositis and dermatomyositis","volume":"292","author":"Bohan","year":"1975","journal-title":"N Engl J Med"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2004.02.006","article-title":"119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10-12 October 2003, Naarden, The Netherlands","volume":"14","author":"Hoogendijk","year":"2004","journal-title":"Neuromuscul Disord"},{"key":"B6","doi-asserted-by":"publisher","first-page":"817922143602251384098","DOI":"10.1177\/22143602251384098","article-title":"Emerging therapies in idiopathic inflammatory myopathies","volume":"13","author":"Kocyigit","year":"2025","journal-title":"J Neuromuscular Dis"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1128\/cmr.00001-08","article-title":"Bacterial, fungal, parasitic, and viral myositis","volume":"21","author":"Crum-Cianflone","year":"2008","journal-title":"Clin Microbiol Rev"},{"key":"B8","doi-asserted-by":"publisher","first-page":"486","DOI":"10.1038\/s41418-017-0012-4","article-title":"Molecular mechanisms of cell death: recommendations of the Nomenclature Committee on Cell Death 2018","volume":"25","author":"Galluzzi","year":"2018","journal-title":"Cell Death Differ"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.3390\/ijms24010362","article-title":"Mechanisms of myofibre death in muscular dystrophies: the emergence of the regulated forms of necrosis in myology","volume":"24","author":"Bencze","year":"2023","journal-title":"Int J Mol Sci"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1002\/art.42071","article-title":"Contribution of necroptosis to myofiber death in idiopathic inflammatory myopathies","volume":"74","author":"Peng","year":"2022","journal-title":"Arthritis Rheumatol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"3655","DOI":"10.1038\/s41467-018-06057-9","article-title":"Necroptosis mediates myofibre death in dystrophin-deficient mice","volume":"9","author":"Morgan","year":"2018","journal-title":"Nat Commun"},{"key":"B12","doi-asserted-by":"publisher","first-page":"8497","DOI":"10.3390\/ijms24108497","article-title":"Inflammasome activity in the skeletal muscle and heart of rodent models for Duchenne muscular dystrophy","volume":"24","author":"On\u00f3di","year":"2023","journal-title":"Int J Mol Sci"},{"key":"B13","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1038\/s41467-021-27875-4","article-title":"Targeting necroptosis in muscle fibers ameliorates inflammatory myopathies","volume":"13","author":"Kamiya","year":"2022","journal-title":"Nat Commun"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1242\/dmm.018127","article-title":"Increased autophagy and apoptosis contribute to muscle atrophy in a myotonic dystrophy type 1 Drosophila model","volume":"8","author":"Bargiela","year":"2015","journal-title":"Dis Model Mech"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1038\/cdd.2010.33","article-title":"Normal myogenesis and increased apoptosis in myotonic dystrophy type-1 muscle cells","volume":"17","author":"Loro","year":"2010","journal-title":"Cell Death Differ"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/nlaa023","article-title":"Gasdermine E-dependent mitochondrial pyroptotic pathway in dermatomyositis: a possible mechanism of perifascicular atrophy","volume":"79","author":"Liu","year":"2020","journal-title":"J Neuropathol Exp Neurol"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1016\/j.it.2020.10.005","article-title":"Coronaviruses: innate immunity, inflammasome activation, inflammatory cell death, and cytokines","volume":"41","author":"Lee","year":"2020","journal-title":"Trends Immunol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1016\/j.it.2024.02.004","article-title":"Inflammasome diversity: exploring novel frontiers in the innate immune response","volume":"45","author":"Yu","year":"2024","journal-title":"Trends Immunol"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1038\/nature15514","article-title":"Cleavage of GSDMD by inflammatory caspases determines pyroptotic cell death","volume":"526","author":"Shi","year":"2015","journal-title":"Nature"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1038\/nature18629","article-title":"Inflammasome-activated gasdermin D causes pyroptosis by forming membrane pores","volume":"535","author":"Liu","year":"2016","journal-title":"Nature"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1038\/nature18590","article-title":"Pore-forming activity and structural autoinhibition of the gasdermin family","volume":"535","author":"Ding","year":"2016","journal-title":"Nature"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1038\/s41422-020-0295-8","article-title":"Toward targeting inflammasomes: insights into their regulation and activation","volume":"30","author":"Christgen","year":"2020","journal-title":"Cell Res"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1126\/sciimmunol.abo6294","article-title":"ZBP1-dependent inflammatory cell death, PANoptosis, and cytokine storm disrupt IFN therapeutic efficacy during coronavirus infection","volume":"7","author":"Karki","year":"2022","journal-title":"Sci Immunol"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2023.1148727","article-title":"Recent advances in ZBP1-derived PANoptosis against viral infections","volume":"14","author":"Oh","year":"2023","journal-title":"Front Immunol"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1016\/j.mib.2020.07.012","article-title":"PANoptosis in microbial infection","volume":"59","author":"Place","year":"2021","journal-title":"Curr Opin Microbiol"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.4049\/immunohorizons.2100059","article-title":"Inflammatory cell death, PANoptosis, mediated by cytokines in diverse cancer lineages inhibits tumor growth","volume":"5","author":"Malireddi","year":"2021","journal-title":"ImmunoHorizons"},{"key":"B27","doi-asserted-by":"publisher","first-page":"3151","DOI":"10.1038\/s41467-020-16887-1","article-title":"MLKL trafficking and accumulation at the plasma membrane control the kinetics and threshold for necroptosis","volume":"11","author":"Samson","year":"2020","journal-title":"Nat Commun"},{"key":"B28","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1080\/01926230701320337","article-title":"Apoptosis: a review of programmed cell death","volume":"35","author":"Elmore","year":"2007","journal-title":"Toxicol Pathol"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1016\/s0092-8674(00)80434-1","article-title":"Cytochrome c and dATP-dependent formation of Apaf-1\/caspase-9 complex initiates an apoptotic protease cascade","volume":"91","author":"Li","year":"1997","journal-title":"Cell"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1995.tb00245.x","article-title":"Cytotoxicity-dependent APO-1 (Fas\/CD95)-associated proteins form a death-inducing signaling complex (DISC) with the receptor","volume":"14","author":"Kischkel","year":"1995","journal-title":"EMBO J"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1126\/science.281.5381.1305","article-title":"Death receptors: signaling and modulation","volume":"281","author":"Ashkenazi","year":"1998","journal-title":"Science"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1038\/nri.2016.150","article-title":"Regulation of muscle growth and regeneration by the immune system","volume":"17","author":"Tidball","year":"2017","journal-title":"Nat Rev Immunol"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.3390\/ijms21124440","article-title":"Progressive skeletal muscle atrophy in muscular dystrophies: a role for toll-like receptor-signaling in disease pathogenesis","volume":"21","author":"De Paepe","year":"2020","journal-title":"Int J Mol Sci"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1152\/ajpregu.2000.279.4.R1165","article-title":"NF-\u03baB mediates the protein loss induced by TNF-\u03b1 in differentiated skeletal muscle myotubes","volume":"279","author":"Li","year":"2000","journal-title":"Am J Physiol Regul Intgr Comp Physiol"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1038\/nri2936","article-title":"Emerging inflammasome effector mechanisms","volume":"11","author":"Lamkanfi","year":"2011","journal-title":"Nat Rev Immunol"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1038\/nm.3893","article-title":"Inflammasomes: mechanism of action, role in disease, and therapeutics","volume":"21","author":"Guo","year":"2015","journal-title":"Nat Med"},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.3390\/cells10113023","article-title":"Walking down skeletal muscle lane: from inflammasome to disease","volume":"10","author":"Dubuisson","year":"2021","journal-title":"Cells"},{"key":"B38","doi-asserted-by":"publisher","DOI":"10.1186\/s40635-016-0115-0","article-title":"Deletion of Nlrp3 protects from inflammation-induced skeletal muscle atrophy","volume":"5","author":"Huang","year":"2017","journal-title":"Intensive Care Med Exp"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.1035709","article-title":"The role of NLRP3 inflammasome in inflammation-related skeletal muscle atrophy","volume":"13","author":"Liu","year":"2022","journal-title":"Front Immunol"},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.7150\/thno.74831","article-title":"Ablation of NLRP3 inflammasome attenuates muscle atrophy via inhibiting pyroptosis, proteolysis and apoptosis following denervation","volume":"13","author":"You","year":"2023","journal-title":"Theranostics"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1126\/science.1065874","article-title":"Identification of ubiquitin ligases required for skeletal muscle atrophy","volume":"294","author":"Bodine","year":"2001","journal-title":"Science"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.00060.2017","article-title":"The NLRP3 inflammasome contributes to sarcopenia and lower muscle glycolytic potential in old mice","volume":"313","author":"McBride","year":"2017","journal-title":"Am J Physiol Endocrinol Metab"},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.1101\/gad.1228704","article-title":"Signaling to NF-kappaB","volume":"18","author":"Hayden","year":"2004","journal-title":"Genes Dev"},{"key":"B44","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2004.09.027","article-title":"IKKbeta\/NF-kappaB activation causes severe muscle wasting in mice","volume":"119","author":"Cai","year":"2004","journal-title":"Cell"},{"key":"B45","doi-asserted-by":"publisher","DOI":"10.1126\/science.289.5488.2363","article-title":"NF-\u03baB-induced loss of MyoD messenger RNA: possible role in muscle decay and cachexia","volume":"289","author":"Guttridge","year":"2000","journal-title":"Science"},{"key":"B46","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1038\/s41420-023-01365-6","article-title":"TNF-\u03b1 contributes to sarcopenia through caspase-8\/caspase-3\/GSDME-mediated pyroptosis","volume":"9","author":"Wu","year":"2023","journal-title":"Cell Death Discov"},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200909117","article-title":"The TWEAK\u2013Fn14 system is a critical regulator of denervation-induced skeletal muscle atrophy in mice","volume":"188","author":"Mittal","year":"2010","journal-title":"J Cell Biol"},{"key":"B48","doi-asserted-by":"publisher","DOI":"10.1038\/ni.1863","article-title":"The role of pattern-recognition receptors in innate immunity: update on toll-like receptors","volume":"11","author":"Kawai","year":"2010","journal-title":"Nat Immunol"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.1152\/ajpregu.00490.2005","article-title":"Multiple toll-like receptor ligands induce an IL-6 transcriptional response in skeletal myocytes","volume":"290","author":"Frost","year":"2006","journal-title":"Am J Physiol Regul Intgr Comp Physiol"},{"key":"B50","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.12491","article-title":"Serum amyloid A1 mediates myotube atrophy via Toll-like receptors","volume":"11","author":"Hahn","year":"2020","journal-title":"J Cachexia Sarcopenia Muscle"},{"key":"B51","doi-asserted-by":"publisher","DOI":"10.1038\/nri1604","article-title":"Mechanisms of type-I- and type-II-interferon-mediated signalling","volume":"5","author":"Platanias","year":"2005","journal-title":"Nat Rev Immunol"},{"key":"B52","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20464","article-title":"Interferon-\u03b1\/\u03b2\u2013mediated innate immune mechanisms in dermatomyositis","volume":"57","author":"Greenberg","year":"2005","journal-title":"Ann Neurol"},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keae082","article-title":"Genetic changes from type I interferons and JAK inhibitors: clues to drivers of juvenile dermatomyositis","volume":"63","author":"Covert","year":"2024","journal-title":"Rheumatology"},{"key":"B54","doi-asserted-by":"publisher","DOI":"10.1111\/febs.12338","article-title":"Interleukin-6 myokine signaling in skeletal muscle: a double-edged sword","volume":"280","author":"Mu\u00f1oz-C\u00e1noves","year":"2013","journal-title":"FEBS J"},{"key":"B55","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1155\/2014\/206026","article-title":"IL-6 impairs myogenic differentiation by downmodulation of p90RSK\/eEF2 and mTOR\/p70S6K axes, without affecting AKT activity","volume":"2014","author":"Michele Pelosi","year":"2014","journal-title":"BioMed Res Int"},{"key":"B56","doi-asserted-by":"publisher","DOI":"10.1002\/art.24689","article-title":"Therapeutic effects of interleukin-6 blockade in a murine model of polymyositis that does not require interleukin-17A","volume":"60","author":"Okiyama","year":"2009","journal-title":"Arthritis Rheumatism"},{"key":"B57","doi-asserted-by":"publisher","DOI":"10.1172\/JCI118398","article-title":"Interleukin 6 receptor antibody inhibits muscle atrophy and modulates proteolytic systems in interleukin 6 transgenic mice","volume":"97","author":"Tsujinaka","year":"1996","journal-title":"J Clin Invest"},{"key":"B58","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2004.032359","article-title":"Contribution of tumour necrosis factor \u03b1 and interleukin (IL) 1\u03b2 to IL6 production, NF-\u03baB nuclear translocation, and class I MHC expression in muscle cells: in vitro regulation with specific cytokine inhibitors","volume":"64","author":"Chevrel","year":"2005","journal-title":"Ann Rheumatic Dis"},{"key":"B59","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-5728(03)00032-8","article-title":"Interleukin-17 increases the effects of IL-1\u03b2 on muscle cells: arguments for the role of T cells in the pathogenesis of myositis","volume":"137","author":"Chevrel","year":"2003","journal-title":"J Neuroimmunology"},{"key":"B60","doi-asserted-by":"publisher","DOI":"10.1093\/intimm\/dxf062","article-title":"Increased IL\u201015 production of muscle cells in polymyositis and dermatomyositis","volume":"14","author":"Sugiura","year":"2002","journal-title":"Int Immunol"},{"key":"B61","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2016.02.015","article-title":"Macrophages in tissue repair, regeneration, and fibrosis","volume":"44","author":"Wynn","year":"2016","journal-title":"Immunity"},{"key":"B62","doi-asserted-by":"publisher","first-page":"8179","DOI":"10.3390\/ijms22158179","article-title":"Mitochondrial dynamics and mitophagy in skeletal muscle health and aging","volume":"22","author":"Leduc-Gaudet","year":"2021","journal-title":"Int J Mol Sci"},{"key":"B63","doi-asserted-by":"publisher","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":"B64","doi-asserted-by":"publisher","first-page":"705","DOI":"10.1016\/j.ncl.2014.04.011","article-title":"Myotonic dystrophy","volume":"32","author":"Thornton","year":"2014","journal-title":"Neurol Clin"},{"key":"B65","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2015.09.007","article-title":"Expansion size and presence of CCG\/CTC\/CGG sequence interruptions in the expanded CTG array are independently associated to hypermethylation at the DMPK locus in myotonic dystrophy type 1 (DM1)","volume":"1852","author":"Santoro","year":"2015","journal-title":"Biochim Biophys Acta (BBA) - Mol Basis Dis"},{"key":"B66","doi-asserted-by":"publisher","DOI":"10.3389\/fgene.2022.865811","article-title":"Expanded CUG repeat RNA induces premature senescence in myotonic dystrophy model cells","volume":"13","author":"Hasuike","year":"2022","journal-title":"Front Genet"},{"key":"B67","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.159357","article-title":"Senescence plays a role in myotonic dystrophy type 1","volume":"7","author":"Garc\u00eda-Puga","year":"2022","journal-title":"JCI Insight"},{"key":"B68","doi-asserted-by":"publisher","first-page":"4033","DOI":"10.1038\/s41467-023-39663-3","article-title":"Clearance of defective muscle stem cells by senolytics restores myogenesis in myotonic dystrophy type 1","volume":"14","author":"Conte","year":"2023","journal-title":"Nat Commun"},{"key":"B69","doi-asserted-by":"publisher","first-page":"16","DOI":"10.1111\/ncn3.12687","article-title":"Expanded-repeat-RNA-mediated disease mechanisms in myotonic dystrophy","volume":"12","author":"Nakamori","year":"2024","journal-title":"Neurol Clin Neurosci"},{"key":"B70","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddu617","article-title":"TWEAK\/Fn14, a pathway and novel therapeutic target in myotonic dystrophy","volume":"24","author":"Yadava","year":"2014","journal-title":"Hum Mol Genet"},{"key":"B71","doi-asserted-by":"publisher","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":"2011","journal-title":"Hum Mol Genet"},{"key":"B72","doi-asserted-by":"publisher","first-page":"1534","DOI":"10.1038\/s41467-024-45535-1","article-title":"Chronic endoplasmic reticulum stress in myotonic dystrophy type 2 promotes autoimmunity via mitochondrial DNA release","volume":"15","author":"R\u00f6sing","year":"2024","journal-title":"Nat Commun"},{"key":"B73","doi-asserted-by":"publisher","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":"B74","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2017.10.018","article-title":"Aberrant myokine signaling in congenital myotonic dystrophy","volume":"21","author":"Nakamori","year":"2017","journal-title":"Cell Rep"},{"key":"B75","article-title":"Apoptotic myonuclei in human Duchenne muscular dystrophy","volume":"78","author":"Sandri","year":"1998","journal-title":"Lab Invest"},{"key":"B76","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-002-0635-4","article-title":"Expression of tumor necrosis factor-\u03b1 in regenerating muscle fibers in inflammatory and non-inflammatory myopathies","volume":"105","author":"Kuru","year":"2003","journal-title":"Acta Neuropathol"},{"key":"B77","first-page":"16","article-title":"Activation of NF-kappaB pathway in Duchenne muscular dystrophy: relation to age","volume":"30","author":"Messina","year":"2011","journal-title":"Acta Myol"},{"key":"B78","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddu735","article-title":"Toll-like receptor 4 ablation in mdx mice reveals innate immunity as a therapeutic target in Duchenne muscular dystrophy","volume":"24","author":"Giordano","year":"2014","journal-title":"Hum Mol Genet"},{"key":"B79","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1507719112","article-title":"Large-scale serum protein biomarker discovery in Duchenne muscular dystrophy","volume":"112","author":"Hathout","year":"2015","journal-title":"Proc Natl Acad Sci"},{"key":"B80","doi-asserted-by":"publisher","DOI":"10.1016\/j.ebiom.2015.02.014","article-title":"Functional and morphological improvement of dystrophic muscle by interleukin 6 receptor blockade","volume":"2","author":"Pelosi","year":"2015","journal-title":"eBioMedicine"},{"key":"B81","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1186\/s13395-017-0140-z","article-title":"Treatment with the anti-IL-6 receptor antibody attenuates muscular dystrophy via promoting skeletal muscle regeneration in dystrophin-\/utrophin-deficient mice","volume":"7","author":"Wada","year":"2017","journal-title":"Skeletal Muscle"},{"key":"B82","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2023.1119888","article-title":"Growth hormone secretagogues modulate inflammation and fibrosis in mdx mouse model of Duchenne muscular dystrophy","volume":"14","author":"Boccanegra","year":"2023","journal-title":"Front Immunol"},{"key":"B83","doi-asserted-by":"publisher","DOI":"10.1016\/0049-0172(86)90014-4","article-title":"Adult onset polymyositis\/dermatomyositis: an analysis of clinical and laboratory features and survival in 76 patients with a review of the literature","volume":"15","author":"Hochberg","year":"1986","journal-title":"Semin Arthritis Rheumatism"},{"key":"B84","doi-asserted-by":"publisher","DOI":"10.1016\/S0733-8619(05)70337-6","article-title":"Idiopathic inflammatory myopathies","volume":"15","author":"Amato","year":"1997","journal-title":"Neurologic Clinics"},{"key":"B85","doi-asserted-by":"publisher","DOI":"10.1097\/00002281-199911000-00005","article-title":"Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis","volume":"11","author":"Sontheimer","year":"1999","journal-title":"Curr Opin Rheumatol"},{"key":"B86","doi-asserted-by":"publisher","first-page":"4301","DOI":"10.3390\/ijms23084301","article-title":"Pathophysiological mechanisms and treatment of dermatomyositis and immune mediated necrotizing myopathies: a focused review","volume":"23","author":"Kamperman","year":"2022","journal-title":"Int J Mol Sci"},{"key":"B87","doi-asserted-by":"publisher","first-page":"198","DOI":"10.1007\/s11926-010-0101-6","article-title":"Dermatomyositis and type 1 interferons","volume":"12","author":"Greenberg","year":"2010","journal-title":"Curr Rheumatol Rep"},{"key":"B88","doi-asserted-by":"publisher","first-page":"5","DOI":"10.3233\/jnd-230168","article-title":"Contribution of complement, microangiopathy and inflammation in idiopathic inflammatory myopathies","volume":"11","author":"Honda","year":"2024","journal-title":"J Neuromuscular Dis"},{"key":"B89","doi-asserted-by":"publisher","DOI":"10.4103\/0366-6999.180528","article-title":"Increased expression of the NOD-like receptor family, pyrin domain containing 3 inflammasome in dermatomyositis and polymyositis is a potential contributor to their pathogenesis","volume":"129","author":"Yin","year":"2016","journal-title":"Chin Med J"},{"key":"B90","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaa473","article-title":"PKM2-dependent glycolysis promotes skeletal muscle cell pyroptosis by activating the NLRP3 inflammasome in dermatomyositis\/polymyositis","volume":"60","author":"Liu","year":"2020","journal-title":"Rheumatology"},{"key":"B91","doi-asserted-by":"publisher","DOI":"10.1097\/NEN.0b013e31826bf7f3","article-title":"Toll-like receptors promote inflammation in idiopathic inflammatory myopathies","volume":"71","author":"Brunn","year":"2012","journal-title":"J Neuropathol Exp Neurol"},{"key":"B92","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31821f440a","article-title":"Type I interferon and Toll-like receptor expression characterizes inflammatory myopathies","volume":"76","author":"Cappelletti","year":"2011","journal-title":"Neurology"},{"key":"B93","doi-asserted-by":"publisher","DOI":"10.1002\/art.27465","article-title":"Expression of toll-like receptor 3 and toll-like receptor 7 in muscle is characteristic of inflammatory myopathy and is differentially regulated by Th1 and Th17 cytokines","volume":"62","author":"Tournadre","year":"2010","journal-title":"Arthritis Rheumatism"},{"key":"B94","doi-asserted-by":"publisher","first-page":"190","DOI":"10.1002\/art.42989","article-title":"TLR7\/8 activation in immune cells and muscle by RNA-containing immune complexes: role in inflammation and the pathogenesis of myositis","volume":"77","author":"Wu","year":"2025","journal-title":"Arthritis Rheumatol"},{"key":"B95","article-title":"Dual effects of caspase-1, interleukin-1 beta, tumour necrosis factor-alpha and nerve growth factor receptor in inflammatory myopathies","volume":"21","author":"Mackiewicz","year":"2003","journal-title":"Clin Exp Rheumatol"},{"key":"B96","doi-asserted-by":"publisher","first-page":"136","DOI":"10.1186\/s13075-016-1033-y","article-title":"Effects on muscle tissue remodeling and lipid metabolism in muscle tissue from adult patients with polymyositis or dermatomyositis treated with immunosuppressive agents","volume":"18","author":"Loell","year":"2016","journal-title":"Arthritis Res Ther"},{"key":"B97","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-021-03875-8","article-title":"AIM2 forms a complex with pyrin and ZBP1 to drive PANoptosis and host defence","volume":"597","author":"Lee","year":"2021","journal-title":"Nature"},{"key":"B98","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1212\/WNL.0000000000003568","article-title":"Sarcoplasmic MxA expression","volume":"88","author":"Uruha","year":"2017","journal-title":"Neurology"},{"key":"B99","doi-asserted-by":"publisher","DOI":"10.1126\/sciimmunol.aau4643","article-title":"Influenza restriction factor MxA functions as inflammasome sensor in the respiratory epithelium","volume":"4","author":"Lee","year":"2019","journal-title":"Sci Immunol"},{"key":"B100","doi-asserted-by":"publisher","DOI":"10.1002\/art.34659","article-title":"Changes in novel biomarkers of disease activity in juvenile and adult dermatomyositis are sensitive biomarkers of disease course","volume":"64","author":"Reed","year":"2012","journal-title":"Arthritis Rheum"},{"key":"B101","doi-asserted-by":"publisher","DOI":"10.1002\/art.24936","article-title":"Interleukin-6 and type I interferon\u2013regulated genes and chemokines mark disease activity in dermatomyositis","volume":"60","author":"Bilgic","year":"2009","journal-title":"Arthritis Rheumatism"},{"key":"B102","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000200271","article-title":"Involvement of type I interferon signaling in muscle stem cell proliferation during dermatomyositis","volume":"98","author":"Gallay","year":"2022","journal-title":"Neurology"},{"key":"B103","doi-asserted-by":"publisher","DOI":"10.1016\/j.ard.2026.02.018","article-title":"Inhibition of the type I interferon receptor pathway protects against muscle weakness induced by dermatomyositis serum","volume":"85","author":"Kaewin","year":"2026","journal-title":"Ann Rheumatic Dis"},{"key":"B104","doi-asserted-by":"publisher","DOI":"10.1007\/s00296-017-3752-z","article-title":"Serum levels of adipokines in patients with idiopathic inflammatory myopathies: a pilot study","volume":"37","author":"Loaiza-F\u00e9lix","year":"2017","journal-title":"Rheumatol Int"},{"key":"B105","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2011-200495","article-title":"Effects of immunosuppressive treatment on interleukin-15 and interleukin-15 receptor \u03b1 expression in muscle tissue of patients with polymyositis or dermatomyositis","volume":"71","author":"Zong","year":"2012","journal-title":"Ann Rheumatic Dis"},{"key":"B106","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2020.12603","article-title":"Persistent symptoms in patients after acute COVID-19","volume":"324","author":"Carf\u00ec","year":"2020","journal-title":"JAMA"},{"key":"B107","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2021.2004","article-title":"Association between SARS-CoV-2 infection and immune-mediated myopathy in patients who have died","volume":"78","author":"Aschman","year":"2021","journal-title":"JAMA Neurol"},{"key":"B108","doi-asserted-by":"publisher","DOI":"10.1111\/ene.14564","article-title":"Muscle involvement in SARS-CoV-2 infection","volume":"28","author":"Pitscheider","year":"2021","journal-title":"Eur J Neurol"},{"key":"B109","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2020.06.003","article-title":"Critical illness myopathy as a consequence of Covid-19 infection","volume":"131","author":"Tankisi","year":"2020","journal-title":"Clin Neurophysiol"},{"key":"B110","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2021.03.016","article-title":"Critical illness polyneuropathy, myopathy and neuronal biomarkers in COVID-19 patients: A prospective study","volume":"132","author":"Frithiof","year":"2021","journal-title":"Clin Neurophysiol"},{"key":"B111","doi-asserted-by":"publisher","DOI":"10.1001\/jamainternmed.2020.6278","article-title":"Diaphragm pathology in critically ill patients with COVID-19 and postmortem findings from 3 medical centers","volume":"181","author":"Shi","year":"2021","journal-title":"JAMA Intern Med"},{"key":"B112","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1186\/s12879-022-07753-0","article-title":"Association of human myxovirus resistance protein A with severity of COVID-19","volume":"22","author":"Lehtinen","year":"2022","journal-title":"BMC Infect Dis"},{"key":"B113","doi-asserted-by":"publisher","DOI":"10.1002\/ana.26318","article-title":"COVID-19-associated critical illness myopathy with direct viral effects","volume":"91","author":"Dodig","year":"2022","journal-title":"Ann Neurol"},{"key":"B114","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00096.2023","article-title":"Cytokine storm in individuals with severe COVID-19 decreases endothelial cell antioxidant defense via downregulation of the Nrf2 transcriptional factor","volume":"325","author":"Rodrigues","year":"2023","journal-title":"Am J Physiol Heart Circ Physiol"},{"key":"B115","doi-asserted-by":"publisher","DOI":"10.1038\/s12276-025-01539-5","article-title":"Prolonged immune activation in post-acute sequelae of SARS-CoV-2: neutrophil dynamics and therapeutic insights","volume":"57","author":"Yu","year":"2025","journal-title":"Exp Mol Med"},{"key":"B116","doi-asserted-by":"publisher","DOI":"10.1093\/mrcr\/rxac041","article-title":"Post-COVID-19 vaccination inflammatory syndrome: A case report","volume":"7","author":"Durucan","year":"2022","journal-title":"Modern Rheumatol Case Rep"},{"key":"B117","doi-asserted-by":"publisher","DOI":"10.3346\/jkms.2022.37.e91","article-title":"Clinicopathological characteristics of inflammatory myositis induced by COVID-19 vaccine (Pfizer-bioNTech BNT162b2): A case report","volume":"37","author":"Kim","year":"2022","journal-title":"jkms"},{"key":"B118","doi-asserted-by":"publisher","DOI":"10.1111\/dth.15749","article-title":"Vaccine-induced dermatomyositis following COVID-19 vaccination","volume":"35","author":"Chaima","year":"2022","journal-title":"Dermatol Ther"},{"key":"B119","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keac038","article-title":"Dermatomyositis-like rash and inflammatory myopathy after mRNA-1273 vaccination","volume":"61","author":"Kondo","year":"2022","journal-title":"Rheumatology"},{"key":"B120","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2021.108765","article-title":"Effective DNA damage response after acute but not chronic immune challenge: SARS-CoV-2 vaccine versus Systemic Lupus Erythematosus","volume":"229","author":"Ntouros","year":"2021","journal-title":"Clin Immunol"},{"key":"B121","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.710647","article-title":"Influenza viruses: innate immunity and mRNA vaccines","volume":"12","author":"Lee","year":"2021","journal-title":"Front Immunol"},{"key":"B122","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1402225","article-title":"Inflammatory muscle diseases","volume":"372","author":"Dalakas","year":"2015","journal-title":"N Engl J Med"},{"key":"B123","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32910-1","article-title":"Muscular dystrophies","volume":"394","author":"Mercuri","year":"2019","journal-title":"Lancet"},{"key":"B124","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1038\/s41420-021-00648-0","article-title":"Trimetazidine attenuates dexamethasone-induced muscle atrophy via inhibiting NLRP3\/GSDMD pathway-mediated pyroptosis","volume":"7","author":"Wang","year":"2021","journal-title":"Cell Death Discov"},{"key":"B125","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.1049076","article-title":"Inhibiting the inflammasome with MCC950 counteracts muscle pyroptosis and improves Duchenne muscular dystrophy","volume":"13","author":"Dubuisson","year":"2022","journal-title":"Front Immunol"},{"key":"B126","doi-asserted-by":"publisher","DOI":"10.1016\/j.intimp.2021.107810","article-title":"Study of the correlation between the noncanonical pathway of pyroptosis and idiopathic inflammatory myopathy","volume":"98","author":"Ma","year":"2021","journal-title":"Int Immunopharmacol"},{"key":"B127","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22158057","article-title":"Dieckol attenuated glucocorticoid-induced muscle atrophy by decreasing NLRP3 inflammasome and pyroptosis","volume":"22","author":"Oh","year":"2021","journal-title":"Int J Mol Sci"},{"key":"B128","doi-asserted-by":"publisher","DOI":"10.71150\/jm.2503009","article-title":"Targeting innate immune sensors for therapeutic strategies in infectious diseases","volume":"63","author":"Shin","year":"2025","journal-title":"J Microbiol"},{"key":"B129","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.12662","article-title":"Amelioration of diabetes-induced inflammation mediated pyroptosis, sarcopenia, and adverse muscle remodelling by bone morphogenetic protein-7","volume":"12","author":"Aluganti Narasimhulu","year":"2021","journal-title":"J Cachexia Sarcopenia Muscle"},{"key":"B130","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2022.10.055","article-title":"JAK inhibitors for the treatment of adult dermatomyositis: A pilot study","volume":"88","author":"Landon-Cardinal","year":"2023","journal-title":"J Am Acad Dermatol"},{"key":"B131","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc1412997","article-title":"Remission of recalcitrant dermatomyositis treated with ruxolitinib","volume":"371","author":"Hornung","year":"2014","journal-title":"N Engl J Med"},{"key":"B132","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2022.08.025","article-title":"Baricitinib for the treatment of cutaneous dermatomyositis: A prospective, open-label study","volume":"87","author":"Zhao","year":"2022","journal-title":"J Am Acad Dermatol"},{"key":"B133","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2016.0866","article-title":"Tofacitinib citrate for refractory cutaneous dermatomyositis: an alternative treatment","volume":"152","author":"Kurtzman","year":"2016","journal-title":"JAMA Dermatol"},{"key":"B134","doi-asserted-by":"publisher","DOI":"10.1002\/art.41602","article-title":"Study of tofacitinib in refractory dermatomyositis: an open-label pilot study of ten patients","volume":"73","author":"Paik","year":"2021","journal-title":"Arthritis Rheumatol"},{"key":"B135","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2012-202794","article-title":"A phase 1b clinical trial evaluating sifalimumab, an anti-IFN-\u03b1 monoclonal antibody, shows target neutralisation of a type I IFN signature in blood of dermatomyositis and polymyositis patients","volume":"73","author":"Higgs","year":"2014","journal-title":"Ann Rheum Dis"},{"key":"B136","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1186\/s13075-020-02164-5","article-title":"Retrospective analysis of infliximab and adalimumab treatment in a large cohort of juvenile dermatomyositis patients","volume":"22","author":"Campanilho-Marques","year":"2020","journal-title":"Arthritis Res Ther"},{"key":"B137","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ken074","article-title":"Effectiveness of infliximab in the treatment of refractory juvenile dermatomyositis with calcinosis","volume":"47","author":"Riley","year":"2008","journal-title":"Rheumatology"},{"key":"B138","doi-asserted-by":"publisher","DOI":"10.1159\/000070852","article-title":"Successful treatment of dermatomyositis and polymyositis with anti-tumor-necrosis-factor-alpha: preliminary observations","volume":"50","author":"Hengstman","year":"2003","journal-title":"Eur Neurol"},{"key":"B139","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2017.10.010","article-title":"A randomized, double-blind, placebo-controlled trial of infliximab in refractory polymyositis and dermatomyositis","volume":"47","author":"Schiffenbauer","year":"2018","journal-title":"Semin Arthritis Rheumatism"},{"key":"B140","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2021.635654","article-title":"The efficacy of tocilizumab in the treatment of patients with refractory immune-mediated necrotizing myopathies: an open-label pilot study","volume":"12","author":"Li","year":"2021","journal-title":"Front Pharmacol"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1871966\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T05:59:22Z","timestamp":1783490362000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1871966\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,8]]},"references-count":140,"alternative-id":["10.3389\/fimmu.2026.1871966"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2026.1871966","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7,8]]},"article-number":"1871966"}}