{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T11:42:57Z","timestamp":1775043777614,"version":"3.50.1"},"reference-count":70,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2026,3,28]],"date-time":"2026-03-28T00:00:00Z","timestamp":1774656000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Laboratory of the Transmission, Control"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Muscle biopsy (MB) is an important tool to help differentiate idiopathic inflammatory myopathies (IIMs) from hereditary muscular diseases (HMDs). The usefulness of immunohistochemical stains of the major histocompatibility complex class I and the membrane attack complex are controversial, as both may be identified in some HMDs. More sensitive markers of IIMs have recently been used, such as myxovirus resistance A (MxA), a type I interferon-inducible protein. We selected skeletal MB samples from 81 patients diagnosed with IIM and HMD harbouring overt inflammatory infiltrates on their MBs in the period between March 2022 and September 2024. Two groups were identified: the IIM group (46 cases) and the HMD group (35 cases). We characterized and compared the patterns of MxA protein expression among the two groups. In the IIM group, positive sarcoplasmic MxA expression was detected on the myofibres of 10 patients (24%), among whom were eight dermatomyositis patients. In the HMD group, we did not identify any sarcoplasmic positivity. However, five patients (14%) showed positive labelling restricted to the sarcolemmal membrane, including non-necrotic or regenerating fibres. Our study demonstrates the value of MxA for increasing dermatomyositis diagnostic accuracy and suggests the potential role of interferon type I in the pathophysiology of HMD.<\/jats:p>","DOI":"10.3390\/ijms27073091","type":"journal-article","created":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T08:22:36Z","timestamp":1774858956000},"page":"3091","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Myxovirus Resistance A Protein Expression in Idiopathic Inflammatory Myopathies and Hereditary Muscle Diseases with Inflammatory Cell Infiltration: A North African Study"],"prefix":"10.3390","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-3431-4566","authenticated-orcid":false,"given":"Emna","family":"Farhat","sequence":"first","affiliation":[{"name":"Faculty of Medicine of Tunis, Tunis El Manar University, Tunis 1007, Tunisia"},{"name":"Laboratory of the Transmission, Control and Immunobiology of Infections, Pasteur Institute, Tunis 1002, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0438-6807","authenticated-orcid":false,"given":"Imen","family":"Zamali","sequence":"additional","affiliation":[{"name":"Faculty of Medicine of Tunis, Tunis El Manar University, Tunis 1007, Tunisia"},{"name":"Laboratory of the Transmission, Control and Immunobiology of Infections, Pasteur Institute, Tunis 1002, Tunisia"},{"name":"Department of Clinical Immunology, Pasteur Institute of Tunis, Tunis 1002, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thouraya Ben","family":"Younes","sequence":"additional","affiliation":[{"name":"Faculty of Medicine of Tunis, Tunis El Manar University, Tunis 1007, Tunisia"},{"name":"Department of Paediatric Neurology, National Institute Mongi Ben Hamida of Neurology, Tunis 1007, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hedia","family":"Klaa","sequence":"additional","affiliation":[{"name":"Faculty of Medicine of Tunis, Tunis El Manar University, Tunis 1007, Tunisia"},{"name":"Department of Paediatric Neurology, National Institute Mongi Ben Hamida of Neurology, Tunis 1007, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Werner","family":"Stenzel","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Charit\u00e9 Medicine University, 10117 Berlin, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-0652-6484","authenticated-orcid":false,"given":"Samar","family":"Samoud","sequence":"additional","affiliation":[{"name":"Laboratory of the Transmission, Control and Immunobiology of Infections, Pasteur Institute, Tunis 1002, Tunisia"},{"name":"Department of Clinical Immunology, Pasteur Institute of Tunis, Tunis 1002, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hanen","family":"Ben Rhouma","sequence":"additional","affiliation":[{"name":"Faculty of Medicine of Tunis, Tunis El Manar University, Tunis 1007, Tunisia"},{"name":"Department of Paediatric Neurology, National Institute Mongi Ben Hamida of Neurology, Tunis 1007, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yousr","family":"Galai","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology, Pasteur Institute of Tunis, Tunis 1002, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilhem","family":"Ben Youssef-Turki","sequence":"additional","affiliation":[{"name":"Faculty of Medicine of Tunis, Tunis El Manar University, Tunis 1007, Tunisia"},{"name":"Department of Paediatric Neurology, National Institute Mongi Ben Hamida of Neurology, Tunis 1007, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ichraf","family":"Kraoua","sequence":"additional","affiliation":[{"name":"Faculty of Medicine of Tunis, Tunis El Manar University, Tunis 1007, Tunisia"},{"name":"Department of Paediatric Neurology, National Institute Mongi Ben Hamida of Neurology, Tunis 1007, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u00e9lika","family":"Ben Ahmed","sequence":"additional","affiliation":[{"name":"Faculty of Medicine of Tunis, Tunis El Manar University, Tunis 1007, Tunisia"},{"name":"Laboratory of the Transmission, Control and Immunobiology of Infections, Pasteur Institute, Tunis 1002, Tunisia"},{"name":"Department of Clinical Immunology, Pasteur Institute of Tunis, Tunis 1002, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9766-920X","authenticated-orcid":false,"given":"Ahlem","family":"Ben Hmid","sequence":"additional","affiliation":[{"name":"Faculty of Medicine of Tunis, Tunis El Manar University, Tunis 1007, Tunisia"},{"name":"Laboratory of the Transmission, Control and Immunobiology of Infections, Pasteur Institute, Tunis 1002, Tunisia"},{"name":"Department of Clinical Immunology, Pasteur Institute of Tunis, Tunis 1002, Tunisia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,3,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1097\/WCO.0000000000000855","article-title":"Where are we moving in the classification of idiopathic inflammatory myopathies?","volume":"33","author":"Tanboon","year":"2020","journal-title":"Curr. Opin. Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1528","DOI":"10.1001\/jamaneurol.2018.2598","article-title":"Development of a new classification system for idiopathic inflammatory myopathies based on clinical manifestations and myositis-specific autoantibodies","volume":"75","author":"Mariampillai","year":"2018","journal-title":"JAMA Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1097\/BOR.0000000000000536","article-title":"The clinico-serological spectrum of overlap myositis","volume":"30","author":"Fredi","year":"2018","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Giannini, M., Ellezam, B., Leclair, V., Lefebvre, F., Troyanov, Y., Hudson, M., Sen\u00e9cal, J.-L., Geny, B., Landon-Cardinal, O., and Meyer, A. (2023). Scleromyositis: A distinct novel entity within the systemic sclerosis and autoimmune myositis spectrum. Implications for care and pathogenesis. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.974078"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2025","DOI":"10.1016\/S0140-6736(19)32910-1","article-title":"Muscular dystrophies","volume":"394","author":"Mercuri","year":"2019","journal-title":"Lancet"},{"key":"ref_6","unstructured":"Dubowitz, V., Sewry, C.A., and Oldfors, A. (2020). Muscle Biopsy, a Practical Approach, Elsevier. [5th ed.]."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1186\/s13075-024-03458-8","article-title":"Development of differential diagnostic models for distinguishing between limb-girdle muscular dystrophy and idiopathic inflammatory myopathy","volume":"26","author":"Wang","year":"2024","journal-title":"Arthritis Res. Ther."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"e249","DOI":"10.1016\/j.lpm.2010.11.023","article-title":"Myositis or dystrophy? Traps and pitfalls","volume":"40","author":"Benveniste","year":"2011","journal-title":"Presse Med."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"46","DOI":"10.17925\/USN.2023.19.1.46","article-title":"LGMDR1 with prominent limb\u2013joint contractures and inflammatory changes misdiagnosed as scleromyositis with a novel CAPN3 mutation: A case report","volume":"19","author":"Abida","year":"2023","journal-title":"touchREVIEWS Neurol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1097\/BOR.0000000000000441","article-title":"Which nonautoimmune myopathies are most frequently misdiagnosed as myositis?","volume":"29","author":"Mammen","year":"2017","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_11","first-page":"10","article-title":"The inflammatory pathology of dysferlinopathy is distinct from calpainopathy, Becker muscular dystrophy, and inflammatory myopathies","volume":"8","author":"Becker","year":"2022","journal-title":"Acta Neuropathol. Commun."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1007\/s00401-023-02669-8","article-title":"Complement and MHC patterns can provide the diagnostic framework for inflammatory neuromuscular diseases","volume":"147","author":"Nelke","year":"2024","journal-title":"Acta Neuropathol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"3156","DOI":"10.1093\/rheumatology\/kead052","article-title":"The pattern of MHC class I expression in muscle biopsies from patients with myositis and other neuromuscular disorders","volume":"62","author":"Milisenda","year":"2023","journal-title":"Rheumatol. Oxf."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1177\/147323001003800332","article-title":"CD8\/MHC-I complex is specific but not sensitive for the diagnosis of polymyositis","volume":"38","author":"Dai","year":"2010","journal-title":"J. Int. Med. Res."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1025","DOI":"10.1016\/j.nmd.2014.06.436","article-title":"An analysis of the sensitivity and specificity of MHC-I and MHC-II immunohistochemical staining in muscle biopsies for the diagnosis of inflammatory myopathies","volume":"24","author":"Luo","year":"2014","journal-title":"Neuromuscul. Disord."},{"key":"ref_16","first-page":"61","article-title":"The assessment of major histocompatibility complex (MHC) class-I expression in different neuromuscular diseases","volume":"11","author":"Kurdi","year":"2021","journal-title":"Degener. Neurol. Neuromuscul. Dis."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1060","DOI":"10.1093\/jnen\/nlae098","article-title":"Contribution of major histocompatibility complex class II immunostaining in distinguishing idiopathic inflammatory myopathy subgroups: A histopathological cohort study","volume":"83","author":"Lessard","year":"2024","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"e000811","DOI":"10.1136\/rmdopen-2018-000811","article-title":"Distinct interferon signatures stratify inflammatory and dysimmune myopathies","volume":"5","author":"Rigolet","year":"2019","journal-title":"RMD Open"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1002\/ana.21805","article-title":"Interferon stimulated gene 15 (ISG15) conjugates proteins in dermatomyositis muscle with perifascicular atrophy","volume":"67","author":"Salajegheh","year":"2010","journal-title":"Ann. Neurol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1212\/WNL.0000000000003568","article-title":"Sarcoplasmic MxA expression: A valuable marker of dermatomyositis","volume":"88","author":"Uruha","year":"2017","journal-title":"Neurology"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1111\/nan.12519","article-title":"Diagnostic potential of sarcoplasmic MxA expression in subsets of dermatomyositis","volume":"45","author":"Uruha","year":"2019","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.cyto.2014.10.012","article-title":"Interferons as components of the complex web of reactions sustaining inflammation in idiopathic inflammatory myopathies","volume":"74","year":"2015","journal-title":"Cytokine"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1002\/art.40328","article-title":"Myogenic Progenitor Cells Exhibit Type I interferon-driven proangiogenic properties and molecular signature during juvenile dermatomyositis","volume":"70","author":"Gitiaux","year":"2018","journal-title":"Arthritis Rheumatol."},{"key":"ref_24","first-page":"1193","article-title":"Identification of distinctive interferon gene signatures in different types of myositis","volume":"93","author":"Derfoul","year":"2019","journal-title":"Neurology"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1093\/rheumatology\/keu289","article-title":"Incidence and prevalence of inflammatory myopathies: A systematic review","volume":"54","author":"Meyer","year":"2014","journal-title":"Rheumatology"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1016\/0046-8177(89)90128-7","article-title":"Major histocompatibility complex class I antigen expression, immunolocalization of interferon subtypes, and T cell-mediated cytotoxicity in myopathies","volume":"20","author":"Arahata","year":"1989","journal-title":"Hum. Pathol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1038\/nri3581","article-title":"Regulation of type I interferon responses","volume":"14","author":"Ivashkiv","year":"2014","journal-title":"Nat. Rev. Immunol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1146\/annurev-med-051113-024537","article-title":"The JAKSTAT pathway: Impact on human disease and therapeutic intervention","volume":"66","author":"Schwartz","year":"2015","journal-title":"Annu. Rev. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"7776","DOI":"10.1128\/JVI.00546-06","article-title":"Induction of MxA gene expression by influenza A virus requires type I or type III interferon signaling","volume":"81","author":"Holzinger","year":"2007","journal-title":"J. Virol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1089\/jir.2010.0110","article-title":"Interferon-stimulated gene 15 and the protein ISGylation system","volume":"31","author":"Zhang","year":"2011","journal-title":"J. Interferon Cytokine Res."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1002\/ana.20464","article-title":"Interferon alpha\/beta-mediated innate immune mechanisms in dermatomyositis","volume":"57","author":"Greenberg","year":"2005","journal-title":"Ann. Neurol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.cyto.2014.11.026","article-title":"The role of type I interferons and other cytokines in dermatomyositis","volume":"73","author":"Arshanapalli","year":"2015","journal-title":"Cytokine"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1007\/s00401-017-1731-9","article-title":"IFN-\u03b2-induced reactive oxygen species and mitochondrial damage contribute to muscle impairment and inflammation maintenance in dermatomyositis","volume":"134","author":"Meyer","year":"2017","journal-title":"Acta Neuropathol."},{"key":"ref_34","doi-asserted-by":"crossref","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. Neuromuscul. Dis."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1186\/s40478-014-0154-2","article-title":"Myofiber HLA-DR expression is a distinctive biomarker for antisynthetase-associated myopathy","volume":"2","author":"Aouizerate","year":"2014","journal-title":"Acta Neuropathol. Commun."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/j.nmd.2019.10.005","article-title":"239th ENMC international workshop: Classification of dermatomyositis, Amsterdam, The Netherlands, 14\u201316 December 2018","volume":"30","author":"Mammen","year":"2020","journal-title":"Neuromuscul. Disord."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"e739","DOI":"10.1212\/WNL.0000000000013176","article-title":"Dermatomyositis: Muscle pathology according to antibody subtypes","volume":"98","author":"Tanboon","year":"2022","journal-title":"Neurology"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/j.nmd.2024.01.009","article-title":"Amyopathic\u201d MDA5-positive dermatomyositis with severe lung involvement presenting with net myositic morphological features\u2014Insights from an autopsy study","volume":"36","author":"Englert","year":"2024","journal-title":"Neuromuscul. Disord."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1111\/nan.12498","article-title":"Expression of myxovirus-resistance protein A: A possible marker of muscle disease activity and autoantibody specificities in juvenile dermatomyositis","volume":"45","author":"Soponkanaporn","year":"2019","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1002\/mus.28066","article-title":"Myxovirus resistance protein A (MxA) expression in myositides: Sarcoplasmic expression is common in both dermatomyositis and lupus myositis","volume":"69","author":"Xing","year":"2024","journal-title":"Muscle Nerve"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"e13155","DOI":"10.1111\/bpa.13155","article-title":"Muscle pathology of antisynthetase syndrome according to antibody subtypes","volume":"33","author":"Tanboon","year":"2023","journal-title":"Brain Pathol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1111\/nan.12551","article-title":"Absence of sarcoplasmic myxovirus resistance protein A (MxA) expression in antisynthetase syndrome in a cohort of 194 cases","volume":"45","author":"Inoue","year":"2019","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2029","DOI":"10.1136\/ard.2011.150326","article-title":"Patients with systemic lupus erythematosus, myositis, rheumatoid arthritis and scleroderma share activation of a common type I interferon pathway","volume":"70","author":"Higgs","year":"2011","journal-title":"Ann. Rheum. Dis."},{"key":"ref_44","first-page":"575","article-title":"Emerging concepts of type I interferons in SLE pathogenesis and therapy","volume":"18","author":"Psarras","year":"2022","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"2556","DOI":"10.1093\/rheumatology\/keac668","article-title":"Symptomatic myopathies in sarcoidosis: Disease spectrum and myxovirus resistance protein A expression","volume":"62","author":"Chompoopong","year":"2023","journal-title":"Rheumatology"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"256","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":"ref_47","doi-asserted-by":"crossref","first-page":"1609","DOI":"10.1093\/brain\/awy105","article-title":"JAK inhibitor improves type I interferon induced damage: Proof of concept in dermatomyositis","volume":"141","author":"Ladislau","year":"2018","journal-title":"Brain"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"5801","DOI":"10.1093\/rheumatology\/keab116","article-title":"JAK inhibitors are effective in a subset of patients with juvenile dermatomyositis: A monocentric retrospective study","volume":"60","author":"LeVoyer","year":"2021","journal-title":"Rheumatology"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"2537","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":"ref_50","doi-asserted-by":"crossref","unstructured":"Alonso-P\u00e9rez, J., Carrasco-Rozas, A., Borrell-Pages, M., Fern\u00e1ndez-Sim\u00f3n, E., Pi\u00f1ol-Jurado, P., Badimon, L., Wollin, L., Lleix\u00e0, C., Gallardo, E., and Oliv\u00e9, M. (2022). Nintedanib Reduces Muscle Fibrosis and Improves Muscle Function of the Alpha-Sarcoglycan-Deficient Mice. Biomedicines, 10.","DOI":"10.3390\/biomedicines10102629"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1146\/annurev.physiol.010908.163216","article-title":"Mechanisms of muscle degeneration, regeneration, and repair in the muscular dystrophies","volume":"71","author":"Wallace","year":"2009","journal-title":"Annu. Rev. Physiol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1575","DOI":"10.1002\/mus.1192","article-title":"The dystrophin-glycoprotein complex, cellular signaling, and the regulation of cell survival in the muscular dystrophies","volume":"24","author":"Rando","year":"2001","journal-title":"Muscle Nerve"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1152\/ajpcell.00192.2005","article-title":"Impact of sarcoglycan complex on mechanical signal transduction in murine skeletal muscle","volume":"290","author":"Barton","year":"2006","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1007\/s00431-005-1752-6","article-title":"Limb girdle muscular dystrophy type 2I caused by a novel missense mutation in the FKRP gene presenting as acute virus associated myositis in infancy","volume":"165","author":"Kaindl","year":"2006","journal-title":"Eur. J. Pediatr."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1016\/j.ejpn.2007.02.018","article-title":"Inflammation and response to steroid treatment in limb-girdle muscular dystrophy 2I","volume":"11","author":"Darin","year":"2007","journal-title":"Eur. J. Paediatr. Neurol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1111\/j.1749-6632.2011.06220.x","article-title":"Type I Interferonopathies: A Novel Set of Inborn Errors of Immunity","volume":"1238","author":"Crow","year":"2011","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1056\/NEJMoa1312625","article-title":"Activated STING in a Vascular and Pulmonary Syndrome","volume":"371","author":"Liu","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_58","doi-asserted-by":"crossref","unstructured":"Torrelo, A. (2017). CANDLE syndrome as a paradigm of proteasome-related autoinflammation. Front. Immunol., 9.","DOI":"10.3389\/fimmu.2017.00927"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"e12955","DOI":"10.1111\/bpa.12955","article-title":"The role of interferons type I, II and III in myositis: A review","volume":"31","author":"Bolko","year":"2021","journal-title":"Brain Pathology"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1091","DOI":"10.1136\/ard-2023-223873","article-title":"Transcriptional derepression of CHD4\/NuRD-regulated genes in the muscle of patients with dermatomyositis and anti-Mi2 autoantibodies","volume":"82","author":"Milisenda","year":"2023","journal-title":"Ann. Rheum. Dis."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1002\/art.41088","article-title":"Myositis autoantibodies: A comparison of results from the oklahoma medical research foundation myositis panel to the Euroimmun research line blot","volume":"72","author":"Mecoli","year":"2020","journal-title":"Arthritis Rheumatol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1955","DOI":"10.1136\/annrheumdis-2017-211468","article-title":"EULAR\/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups","volume":"76","author":"Lundberg","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1453","DOI":"10.1212\/WNL.0000000000000892","article-title":"Evidence-based guideline summary: Diagnosis and treatment of limb-girdle and distal dystrophies: Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Issues Review Panel of the American Association of Neuromuscular and Electrodiagnostic Medicine","volume":"83","author":"Narayanaswami","year":"2014","journal-title":"Neurology"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1212\/WNL.0000000000001783","article-title":"Evidence-based guideline summary: Evaluation, diagnosis, and management of facioscapulohumeral muscular dystrophy: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the Practice Issues Review Panel of the American Association of Neuromuscular & Electrodiagnostic Medicine","volume":"85","author":"Tawil","year":"2015","journal-title":"Neurology"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1369","DOI":"10.1212\/WNL.0000000000001416","article-title":"Evidence-based guideline summary: Evaluation, diagnosis, and management of congenital muscular dystrophy: Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Issues Review Panel of the American Association of Neuromuscular and Electrodiagnostic Medicine","volume":"84","author":"Kang","year":"2015","journal-title":"Neurology"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/j.nmd.2013.12.011","article-title":"Members of International Standard of Care Committee for Congenital Muscular Dystrophies. Diagnostic approach to the congenital muscular dystrophies","volume":"24","author":"Wang","year":"2014","journal-title":"Neuromuscul. Disord."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1752","DOI":"10.1212\/CON.0000000000001182","article-title":"Metabolic myopathies","volume":"28","author":"Tarnopolsky","year":"2022","journal-title":"Continuum"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1016\/j.nmd.2019.03.002","article-title":"1st ENMC European meeting: The EURO-NMD pathology working group Recommended Standards for Muscle Pathology Amsterdam, The Netherlands, 7 December 2018","volume":"29","author":"Udd","year":"2019","journal-title":"Neuromuscul. Disord."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1192","DOI":"10.1002\/art.23012","article-title":"International consensus on a proposed score system for muscle biopsy evaluation in patients with juvenile dermatomyositis: A tool for potential use in clinical trials","volume":"57","author":"Wedderburn","year":"2007","journal-title":"Arthritis Rheum."},{"key":"ref_70","first-page":"136","article-title":"Diagnostic value of MHC class I staining in idiopathic inflammatory myopathies","volume":"75","author":"Hengstman","year":"2004","journal-title":"J. Neurol. Neurosurg. Psychiat."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/27\/7\/3091\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T10:09:45Z","timestamp":1775038185000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/27\/7\/3091"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,28]]},"references-count":70,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2026,4]]}},"alternative-id":["ijms27073091"],"URL":"https:\/\/doi.org\/10.3390\/ijms27073091","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,28]]}}}