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PXD063841
PXD063841 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Proteomic Profiles in Inclusion Body Myositis and Polymyositis with Mitochondrial Pathology: A Comparative Study Across Idiopathic Inflammatory Myopathies |
| Description | Idiopathic inflammatory myopathies (IIMs) encompass a diverse group of autoimmune disorders affecting skeletal muscle, including dermatomyositis (DM), anti-synthetase syndrome (ASyS), immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). Polymyositis with mitochondrial pathology (PM-Mito) has been proposed as a potential IIM subtype, with emerging evidence suggesting it may represent an early form of IBM. IBM is characterized by unique clinical features, such as insidious onset and resistance to immunotherapies, alongside hallmark histopathological findings, including rimmed vacuoles, cytoplasmic protein aggregates, CD8+ T cell-dominated inflammation, and prominent mitochondrial abnormalities. Mitochondrial dysfunction, ragged-red fibers, and disrupted oxidative phosphorylation are increasingly recognized as central contributors to IBM pathology, implicating mitochondrial processes in both muscle weakness and chronic inflammation. This study presents a longitudinal and cross-sectional proteomic analysis of IBM in comparison to other IIM subtypes. We identified both established and novel hallmarks of IBM, particularly highlighting mitochondrial dysfunction and immune dysregulation, while also examining PM-Mito as a potential precursor state to IBM. Using an unbiased mass spectrometry-based proteomic approach, we identified a distinct proteomic signature for IBM characterized by mitochondrial alterations and type II interferon-associated immune pathway dysregulation. PM-Mito samples displayed a dysregulation of immune regulatory proteins and mitochondrial pathways. This study reinforces the unique molecular profile of IBM compared to other IIM subtypes while strengthening the concept of a disease continuum between IBM and PM-Mito. By uncovering shared mitochondrial and immune dysregulation in IBM and PM-Mito, these findings support the notion that PM-Mito may represent an early or transitional stage of IBM, providing new insights into disease progression and potential therapeutic targets. |
| HostingRepository | jPOST |
| AnnounceDate | 2026-05-12 |
| AnnouncementXML | Submission_2026-05-11_08:00:05.566.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | Ute Distler |
| SpeciesList | scientific name: Homo sapiens (Human); NCBI TaxID: 9606; |
| ModificationList | S-carboxamidomethyl-L-cysteine; alpha-amino acetylated residue; L-methionine sulfoxide |
| Instrument | instrument |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2025-05-11 13:25:37 | ID requested | |
| ⏵ 1 | 2026-05-11 08:00:06 | announced |
Publication List
| Dataset with its publication pending |
Keyword List
| submitter keyword: Idiopathic inflammatory myopathy, label-free proteomics, DIA, inclusion body myositis |
Contact List
| Felix Kleefeld | |
|---|---|
| lab head | |
| Ute Distler | |
| contact affiliation | University Medical Center Mainz |
| dataset submitter | |
Full Dataset Link List
| jPOST dataset URI |
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