ArticleAnnals of neurology2010
Interferon-stimulated gene 15 (ISG15) conjugates proteins in dermatomyositis muscle with perifascicular atrophy.
Article in Annals of neurology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 92 papers.
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Who cites it
92 citing papers in PubMed, 190 citations in OpenAlex.
- Muscle myeloid type I interferon gene expression may predict therapeutic responses to rituximab in myositis patients.Rheumatology (Oxford, England) · 2016Trial
- Suppression of soluble T cell-associated proteins by an anti-interferon-α monoclonal antibody in adult patients with dermatomyositis or polymyositis.Rheumatology (Oxford, England) · 2014Trial
- Interferon-Mediated Regulation of Memory B Cell Identity and Function.Immunological reviews · 2026Review
- Emerging therapies in idiopathic inflammatory myopathies.Journal of neuromuscular diseases · 2026Review
- Article
- Near resolution of cutaneous lesions in long standing refractory dermatomyositis with deucravacitinib: A case report and literature review.JAAD case reports · 2026Article
- Early detection and intervention in dermatomyositis: current evidence and clinical implications.Clinical rheumatology · 2026Review
- Defective plasticity in dermatomyositis patients muscle stem cells is associated with sustained intrinsic inflammatory signaling and disruption of the histone H3.3 chromatin loading pathway.NAR molecular medicine · 2026Article
- Myxovirus Resistance A Protein Expression in Idiopathic Inflammatory Myopathies and Hereditary Muscle Diseases with Inflammatory Cell Infiltration: A North African Study.International journal of molecular sciences · 2026Article
- When ISG15 is involved in inflammation.Frontiers in immunology · 2026Review
- Development of siRNAs targeting interferon beta as a novel therapeutic approach for adult and juvenile dermatomyositis.Frontiers in immunology · 2026Article
- Review
- Risk Factors Associated with the Development of Thrombotic Microangiopathy in Patients with Dermatomyositis.International journal of molecular sciences · 2025Article
- Distinct cytokine and cytokine receptor expression patterns characterize different forms of myositis.Rheumatology (Oxford, England) · 2025Article
- Immune Characterization of a Vietnamese Cohort With Idiopathic Inflammatory Myopathies.Muscle & nerve · 2025Article
- The complement system in autoimmune diseases: pathogenesis, diagnostic markers, and therapeutic strategies.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2025Review
- Construction and validation of a diagnostic model for dermatomyositis based on the LASSO algorithm.Central-European journal of immunology · 2025Article
- Ultrasensitive interferons quantification reveals different cytokine profile secretion in inflammatory myopathies and can serve as biomarkers of activity in dermatomyositis.Frontiers in immunology · 2025Article
- UBXN9 governs GLUT4-mediated spatial confinement of RIG-I-like receptors and signaling.Nature immunology · 2024Article
- Pathological autoantibody internalisation in myositis.Annals of the rheumatic diseases · 2024Article
32 more citing papers are in PubMed but not listed here.
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Authors and funding
15 authors at 3 institutions in 1 country.
Funding
Abstract
objectiveWe investigated interferon-stimulated gene 15 (ISG15), a poorly understood ubiquitin-like modifier, and its enzymatic pathway in dermatomyositis (DM), an autoimmune disease primarily involving muscle and skin.
methodsWe generated microarray data measuring transcript abundance for approximately 18,000 genes in each of 113 human muscle biopsy specimens, and studied biopsy specimens and cultured skeletal muscle using immunohistochemistry, immunoblotting proteomics, real-time quantitative polymerase chain reaction, and laser-capture microdissection.
resultsTranscripts encoding ISG15-conjugation pathway proteins were markedly upregulated in DM with perifascicular atrophy (DM-PFA) muscle (ISG15 339-fold, HERC5 62-fold, and USP18 68-fold) compared with 99 non-DM samples. Combined analysis with publicly available microarray datasets showed that >50-fold ISG15 transcript elevation had 100% sensitivity and specificity for 28 biopsies from adult DM-PFA and juvenile DM patients compared with 199 muscle samples from other muscle diseases. Free ISG15 and ISG15-conjugated proteins were only found on immunoblots from DM-PFA muscle. Cultured human skeletal muscle exposed to type 1 interferons produced similar transcripts and ISG15 protein and conjugates. Laser-capture microdissection followed by proteomic analysis showed deficiency of titin in DM perifascicular atrophic myofibers.
interpretationA large-scale microarray study of muscle samples demonstrated that among a diverse group of muscle diseases DM was uniquely associated with upregulation of the ISG15 conjugation pathway. Exposure of human skeletal muscle cell culture to type 1 interferons produced a molecular picture highly similar to that seen in human DM muscle. Perifascicular atrophic myofibers in DM were deficient in a number of skeletal muscle proteins including titin.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.