Cellular and molecular mechanisms breaking immune tolerance in inborn errors of immunity
| العنوان: | Cellular and molecular mechanisms breaking immune tolerance in inborn errors of immunity |
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| المؤلفون: | Reinhold E. Schmidt, Ignatius Ryan Adriawan, Natalia Dubrowinskaja, Georgios Sogkas, Faranaz Atschekzei, Torsten Witte |
| المصدر: | Cellular and Molecular Immunology |
| بيانات النشر: | Springer Science and Business Media LLC, 2021. |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | Primary immunodeficiencies, Immunology, Receptors, Antigen, T-Cell, Arthritis, Autoimmunity, Review Article, medicine.disease_cause, T-Lymphocytes, Regulatory, Immune tolerance, Rheumatic diseases, Immunity, Immune Tolerance, medicine, Humans, Immunology and Allergy, Genetic Predisposition to Disease, Autoimmune disease, business.industry, Inborn errors of immunity, Diagnostic markers, Immune dysregulation, medicine.disease, Phenotype, Infectious Diseases, Immune System Diseases, Primary immunodeficiency, business, Biomarkers |
| الوصف: | In addition to susceptibility to infections, conventional primary immunodeficiency disorders (PIDs) and inborn errors of immunity (IEI) can cause immune dysregulation, manifesting as lymphoproliferative and/or autoimmune disease. Autoimmunity can be the prominent phenotype of PIDs and commonly includes cytopenias and rheumatological diseases, such as arthritis, systemic lupus erythematosus (SLE), and Sjogren’s syndrome (SjS). Recent advances in understanding the genetic basis of systemic autoimmune diseases and PIDs suggest an at least partially shared genetic background and therefore common pathogenic mechanisms. Here, we explore the interconnected pathogenic pathways of autoimmunity and primary immunodeficiency, highlighting the mechanisms breaking the different layers of immune tolerance to self-antigens in selected IEI. |
| تدمد: | 2042-0226 1672-7681 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::805ca510bf916ab29e4d1a9807fd0821Test https://doi.org/10.1038/s41423-020-00626-zTest |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....805ca510bf916ab29e4d1a9807fd0821 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 20420226 16727681 |
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