دورية أكاديمية
IL-6 and TNF are Potential Inflammatory Biomarkers in Facioscapulohumeral Muscular Dystrophy
| العنوان: | IL-6 and TNF are Potential Inflammatory Biomarkers in Facioscapulohumeral Muscular Dystrophy |
|---|---|
| المؤلفون: | Greco, A., Mul, K., Jaeger, M., Dos Santos, J.C., Koenen, H.J., Jong, L. de, Mann, R.M., Futterer, J.J., Netea, M.G., Pruijn, G.J.M., Engelen, B.G.M. van, Joosten, L.A.B. |
| المصدر: | Journal of Neuromuscular Diseases, 11, 2, pp. 327-347 |
| سنة النشر: | 2024 |
| المجموعة: | Radboud University: DSpace |
| مصطلحات موضوعية: | Bio-Molecular Chemistry, Radboudumc 15: Urological cancers Medical Imaging, Radboudumc 3: Disorders of movement DCMN: Donders Center for Medical Neuroscience, Radboudumc 4: lnfectious Diseases and Global Health Internal Medicine, Radboudumc 5: Inflammatory diseases Laboratory Medicine, Radboudumc 17: Women's cancers Medical Imaging |
| الوصف: | Contains fulltext : 304786.pdf (Publisher’s version ) (Open Access) ; BACKGROUND: FSHD is a highly prevalent inherited myopathy with a still poorly understood pathology. OBJECTIVE: To investigate whether proinflammatory cytokines are associated with FSHD and which specific innate immune cells are involved in its pathology. METHODS: First, we measured circulating cytokines in serum samples: IL-6 (FSHD, n = 150; HC, n = 98); TNF (FSHD, n = 150; HC, n = 59); IL-1α (FSHD, n = 150; HC, n = 66); IL-1β (FSHD, n = 150; HC, n = 98); MCP-1 (FSHD, n = 14; HC, n = 14); VEGF-A (FSHD, n = 14; HC, n = 14). Second, we tested trained immunity in monocytes (FSHD, n = 15; HC, n = 15) and NK cells (FSHD, n = 11; HC, n = 11). Next, we explored the cytokine production capacity of NK cells in response to different stimuli (FSHD, n = 39; HC, n = 22). Lastly, we evaluated the cytokine production of ex vivo stimulated MRI guided inflamed (TIRM+) and paired MRI guided non inflamed (TIRM-) muscle biopsies of 21 patients and of 8 HC muscle biopsies. RESULTS: We included a total of 190 FSHD patients (N = 190, 48±14 years, 49% men) and of 135 HC (N = 135, 44±15 years, 47% men). We found that FSHD patients had higher concentrations of IL-6 and TNF measured (a) in the circulation, (b) after ex-vivo stimulation of NK cells, and (c) in muscle specimens. Besides, IL-6 circulating concentrations, as well as its production by NK cells and IL-6 content of FSHD muscle specimens, showed a mild correlation with disease duration, disease severity, and muscle weakness. CONCLUSION: These results show that IL-6 and TNF may contribute to FSHD pathology and suggest novel therapeutic targets. Additionally, the activation of NK cells in FSHD may be a novel pathway contributing to FSHD pathology. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | unknown |
| العلاقة: | https://repository.ubn.ru.nl//bitstream/handle/2066/304786/304786.pdfTest; https://repository.ubn.ru.nl/handle/2066/304786Test |
| DOI: | 10.3233/JND-230063 |
| الإتاحة: | https://doi.org/10.3233/JND-230063Test https://repository.ubn.ru.nl//bitstream/handle/2066/304786/304786.pdfTest https://repository.ubn.ru.nl/handle/2066/304786Test |
| رقم الانضمام: | edsbas.C8087E88 |
| قاعدة البيانات: | BASE |
| DOI: | 10.3233/JND-230063 |
|---|