{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T16:05:39Z","timestamp":1791216339628,"version":"4.1.0"},"reference-count":40,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,4,29]],"date-time":"2014-04-29T00:00:00Z","timestamp":1398729600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Fondo de Investigaciones Sanitarias","award":["09\/1964"],"award-info":[{"award-number":["09\/1964"]}]}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2014,7]]},"abstract":"Abstract<\/jats:title>We investigated the molecular mechanisms involved in the pathogenesis of three inflammatory myopathies, dermatomyositis (DM<\/jats:styled-content>), polymyositis (PM<\/jats:styled-content>) and inclusion body myositis (IBM<\/jats:styled-content>). We performed microarray experiments\u2020<\/jats:sup> using microdissected pathological muscle fibres from 15 patients with these disorders and five controls. Differentially expressed candidate genes were validated by immunohistochemistry on muscle biopsies, and the altered pathways were analysed in human myotube cultures. Up\u2010regulation of genes involved in viral and nucleic acid recognition were found in the three myopathies but not in controls. In DM<\/jats:styled-content>, retinoic acid\u2010inducible gene 1 (RIG<\/jats:styled-content>\u2010I<\/jats:italic>, DDX58<\/jats:styled-content><\/jats:italic>) and the novel antiviral factor DDX60<\/jats:styled-content><\/jats:italic>, which promotes RIG<\/jats:styled-content>\u2010I\u2010mediated signalling, were significantly up\u2010regulated, followed by IFIH1<\/jats:styled-content><\/jats:italic> (MDA5<\/jats:styled-content><\/jats:italic>) and TLR3<\/jats:styled-content><\/jats:italic>. Immunohistochemistry confirmed over\u2010expression of RIG<\/jats:styled-content>\u2010I in pathological muscle fibres in 5\/5 DM<\/jats:styled-content>, 0\/5 PM<\/jats:styled-content> and 0\/5 IBM<\/jats:styled-content> patients, and in 0\/5 controls. Stimulation of human myotubes with a ligand of RIG<\/jats:styled-content>\u2010I produced a significant secretion of interferon\u2010\u03b2<\/jats:italic> (IFN<\/jats:styled-content>\u03b2<\/jats:italic>; p <<\/jats:italic> 0.05) and up\u2010regulation of class I MHC<\/jats:styled-content>, RIG<\/jats:styled-content>\u2010I and TLR3<\/jats:styled-content> (p <<\/jats:italic> 0.05) by IFN<\/jats:styled-content>\u03b2<\/jats:italic>\u2010dependent and TLR3<\/jats:styled-content>\u2010independent mechanisms. RIG<\/jats:styled-content>\u2010I\u2010mediated innate immunity, triggered by a viral or damage signal, plays a significant role in the pathogenesis of DM<\/jats:styled-content>, but not in that of PM<\/jats:styled-content> or IBM<\/jats:styled-content>. Copyright \u00a9 2014 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd<\/jats:p>","DOI":"10.1002\/path.4346","type":"journal-article","created":{"date-parts":[[2014,3,6]],"date-time":"2014-03-06T10:28:40Z","timestamp":1394101720000},"page":"258-268","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":106,"title":["Altered RIG<\/scp>\u2010I\/DDX58<\/scp>\u2010mediated innate immunity in dermatomyositis"],"prefix":"10.1002","volume":"233","author":[{"given":"Xavier","family":"Su\u00e1rez\u2010Calvet","sequence":"first","affiliation":[{"name":"Neuromuscular Diseases Unit, Neurology Department Hospital de la Santa Creu i Sant Pau, Universitat Aut\u00f2noma de Barcelona and Institut de Recerca Sant Pau Barcelona Spain"},{"name":"Centro Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Neurodegenerativas (CIBERNED) Madrid 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