{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T05:53:11Z","timestamp":1792389191565,"version":"4.4.0"},"reference-count":30,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2023,6,26]],"date-time":"2023-06-26T00:00:00Z","timestamp":1687737600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Association Fran\u00e7aise contre les Myopathies","award":["20987"],"award-info":[{"award-number":["20987"]}]},{"name":"University Medical Center G\u00f6ttingen","award":["20987"],"award-info":[{"award-number":["20987"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Inclusion body myositis (IBM) is a chronic, mostly treatment-resistant, inflammatory myopathy with a pathology that centers around specific interactions between inflammation and protein accumulation. The study aimed to identify the inflammasome as a key event in the complex network of pathomechanisms. Regulation of the inflammasome was assessed in a well-established pro-inflammatory cell culture model using human myoblasts and primary human myotubes. By quantitative PCR, western blot and immunocytochemistry, inflammasome markers including NLRP3 were assessed in muscle cells exposed to the cytokines IL-1\u03b2 and IFN-\u03b3. The data were corroborated by analysis of muscle biopsies from patients with IBM compared to other myositis subtypes. In the cell culture model of IBM, the NLRP3 inflammasome was significantly overexpressed, as evidenced by western blot (p = 0.03) and quantitative PCR (p &lt; 0.01). Target genes that play a role in inflammasome assembly, T-cell migration, and MHC-I expression (p = 0.009) were highly co-upregulated. NLRP3 was significantly overexpressed in muscle biopsies from IBM samples compared to disease controls (p = 0.049), including other inflammatory myopathies. Due to the extraordinary features of the pathogenesis and the pronounced upregulation of NLRP3 in IBM, the inflammasome could serve as a key molecule that drives the inflammatory cascade as well as protein accumulation in the muscle. These data can be useful for future therapeutic developments.<\/jats:p>","DOI":"10.3390\/ijms241310675","type":"journal-article","created":{"date-parts":[[2023,6,27]],"date-time":"2023-06-27T01:42:02Z","timestamp":1687830122000},"page":"10675","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Inflammasome in Skeletal Muscle: NLRP3 Is an Inflammatory Cell Stress Component in Inclusion Body Myositis"],"prefix":"10.3390","volume":"24","author":[{"given":"Karsten","family":"Kummer","sequence":"first","affiliation":[{"name":"Department of Neurology and Pain Treatment, Neuromuscular Center, Center for Translational Medicine, Immanuel Klinik R\u00fcdersdorf, University Hospital of the Brandenburg Medical School, 15562 R\u00fcdersdorf bei Berlin, Germany"},{"name":"Faculty of Health Sciences Brandenburg, Brandenburg Medical School, 15562 R\u00fcdersdorf bei Berlin, Germany"},{"name":"Department of Neurology, Neuromuscular Center, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Imke","family":"Bertram","sequence":"additional","affiliation":[{"name":"Department of Neurology, Neuromuscular Center, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5385-2766","authenticated-orcid":false,"given":"Sabrina","family":"Zechel","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel B.","family":"Hoffmann","sequence":"additional","affiliation":[{"name":"Department of Trauma, Orthopaedic and Plastic Surgery, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Department of Neurology and Pain Treatment, Neuromuscular Center, Center for Translational Medicine, Immanuel Klinik R\u00fcdersdorf, University Hospital of the Brandenburg Medical School, 15562 R\u00fcdersdorf bei Berlin, Germany"},{"name":"Faculty of Health Sciences Brandenburg, Brandenburg Medical School, 15562 R\u00fcdersdorf bei Berlin, Germany"},{"name":"Department of Neurology, Neuromuscular Center, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,6,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"632","DOI":"10.1097\/BOR.0000000000000436","article-title":"Inclusion body myositis: Advancements in diagnosis, pathomechanisms, and treatment","volume":"29","author":"Schmidt","year":"2017","journal-title":"Curr. 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