ArticleFrontiers in immunology2021
Transfer of PBMC From SSc Patients Induces Autoantibodies and Systemic Inflammation in Rag2-/-/IL2rg-/- Mice.
Article in Frontiers in immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 30 citations in OpenAlex.
- Stepwise Evolution of Immunodeficient Mouse Models: Focus on NCG Strains and Their Applications in Humanized Immune System Research.Vaccines · 2026Review
- The application of experimental models for the drug discovery for digestive tumors.Molecular cancer · 2026Review
- Tocilizumab but not Siltuximab prevents systemic inflammation in a humanized mouse model.Frontiers in immunology · 2026Article
- Research advancements and evaluation of multifactor-induced murine models for gastric cancer.Animal models and experimental medicine · 2025Review
- The importance of translational science within the respiratory field.Breathe (Sheffield, England) · 2024Article
- Article
- Peripheral helper-T-cell-derived CXCL13 is a crucial pathogenic factor in idiopathic multicentric Castleman disease.Nature communications · 2023Article
- Transfer of patient's peripheral blood mononuclear cells (PBMCs) disrupts blood-brain barrier and induces anti-NMDAR encephalitis: a study of novel humanized PBMC mouse model.Journal of neuroinflammation · 2023Article
- When natural antibodies become pathogenic: autoantibodies targeted against G protein-coupled receptors in the pathogenesis of systemic sclerosis.Frontiers in immunology · 2023Review
- Murine models to study human NK cells in human solid tumors.Frontiers in immunology · 2023Review
- Autoantibodies as Biomarker and Therapeutic Target in Systemic Sclerosis.Biomedicines · 2022Review
- Establishment of a humanized animal model of systemic sclerosis in which T helper-17 cells from patients with systemic sclerosis infiltrate and cause fibrosis in the lungs and skin.Experimental & molecular medicine · 2022Article
- Both T and B cells are indispensable for the development of a PBMC transfer-induced humanized mouse model for SSc.Arthritis research & therapy · 2022Article
- A broad look into the future of systemic sclerosis.Therapeutic advances in musculoskeletal disease · 2022Review
- The development and improvement of immunodeficient mice and humanized immune system mouse models.Frontiers in immunology · 2022Review
- The Role of B Cells in Scleroderma Lung Disease Pathogenesis.Frontiers in medicine · 2022Review
- Editorial: Precision Medicine in Chronic Inflammation.Frontiers in immunology · 2021Article
Corrections and comments
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Authors and funding
14 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Objective: The contribution of sustained autologous autoantibody production by B cells to the pathogenesis of systemic sclerosis (SSc) and granulomatosis with polyangiitis (GPA) is not fully understood. To investigate this, a humanized mouse model was generated by transferring patient-derived peripheral blood mononuclear cells (PBMC) into immunocompromised mice. Methods: PBMC derived from patients with SSc and GPA as well as healthy controls (HD) were isolated, characterized by flow cytometry, and infused into Results: Mice engrafted with PBMC derived from SSc patients developed autoantibodies such as antinuclear antibodies (ANA) mimicking the pattern of the respective donors. Moreover, cellular infiltrates dominated by B cells were observed in lung, kidney and muscles of the recipient mice. By contrast, PBMC derived from HD or GPA patients survived in recipient mice after transfer, but neither human autoantibodies nor inflammatory infiltrates in tissues were detected. Furthermore, these pathological changes were absent in mice transferred with PBMC from rituximab-treated SSc patients. Conclusion: This humanized mouse model is indicative for cross-reactivity of human lymphocytes to murine autoantigens and argues for a pivotal role of B cells as well as of sustained autoimmunity in the pathogenesis of SSc. It provides a powerful tool to study interstitial lung disease and so far, under-recognized disease manifestations such as myositis and interstitial nephritis.
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