Making Sense of Intracellular Nucleic Acid Sensing in Type I Interferon Activation in Sjögren’s Syndrome
Primary Sjögren’s syndrome (pSS) is a systemic autoimmune rheumatic disease characterized by dryness of the eyes and mucous membranes, which can be accompanied by various extraglandular autoimmune manifestations. The majority of patients exhibit persistent systemic activation of the type I interfero...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-02-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/10/3/532 |
| _version_ | 1797416767717376000 |
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| author | Erika Huijser Marjan A. Versnel |
| author_facet | Erika Huijser Marjan A. Versnel |
| author_sort | Erika Huijser |
| collection | DOAJ |
| description | Primary Sjögren’s syndrome (pSS) is a systemic autoimmune rheumatic disease characterized by dryness of the eyes and mucous membranes, which can be accompanied by various extraglandular autoimmune manifestations. The majority of patients exhibit persistent systemic activation of the type I interferon (IFN) system, a feature that is shared with other systemic autoimmune diseases. Type I IFNs are integral to anti-viral immunity and are produced in response to stimulation of pattern recognition receptors, among which nucleic acid (NA) receptors. Dysregulated detection of endogenous NAs has been widely implicated in the pathogenesis of systemic autoimmune diseases. Stimulation of endosomal Toll-like receptors by NA-containing immune complexes are considered to contribute to the systemic type I IFN activation. Accumulating evidence suggest additional roles for cytosolic NA-sensing pathways in the pathogenesis of systemic autoimmune rheumatic diseases. In this review, we will provide an overview of the functions and signaling of intracellular RNA- and DNA-sensing receptors and summarize the evidence for a potential role of these receptors in the pathogenesis of pSS and the sustained systemic type I IFN activation. |
| first_indexed | 2024-03-09T06:08:58Z |
| format | Article |
| id | doaj.art-f7998d1e89be4743920cd9db49dadae1 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T06:08:58Z |
| publishDate | 2021-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-f7998d1e89be4743920cd9db49dadae12023-12-03T12:00:55ZengMDPI AGJournal of Clinical Medicine2077-03832021-02-0110353210.3390/jcm10030532Making Sense of Intracellular Nucleic Acid Sensing in Type I Interferon Activation in Sjögren’s SyndromeErika Huijser0Marjan A. Versnel1Department of Immunology, Erasmus MC, University Medical Center Rotterdam, 3015 GD Rotterdam, The NetherlandsDepartment of Immunology, Erasmus MC, University Medical Center Rotterdam, 3015 GD Rotterdam, The NetherlandsPrimary Sjögren’s syndrome (pSS) is a systemic autoimmune rheumatic disease characterized by dryness of the eyes and mucous membranes, which can be accompanied by various extraglandular autoimmune manifestations. The majority of patients exhibit persistent systemic activation of the type I interferon (IFN) system, a feature that is shared with other systemic autoimmune diseases. Type I IFNs are integral to anti-viral immunity and are produced in response to stimulation of pattern recognition receptors, among which nucleic acid (NA) receptors. Dysregulated detection of endogenous NAs has been widely implicated in the pathogenesis of systemic autoimmune diseases. Stimulation of endosomal Toll-like receptors by NA-containing immune complexes are considered to contribute to the systemic type I IFN activation. Accumulating evidence suggest additional roles for cytosolic NA-sensing pathways in the pathogenesis of systemic autoimmune rheumatic diseases. In this review, we will provide an overview of the functions and signaling of intracellular RNA- and DNA-sensing receptors and summarize the evidence for a potential role of these receptors in the pathogenesis of pSS and the sustained systemic type I IFN activation.https://www.mdpi.com/2077-0383/10/3/532Sjögren’s syndromeautoimmunecytosolic pattern recognition receptorsnucleic acid sensorsinterferon |
| spellingShingle | Erika Huijser Marjan A. Versnel Making Sense of Intracellular Nucleic Acid Sensing in Type I Interferon Activation in Sjögren’s Syndrome Journal of Clinical Medicine Sjögren’s syndrome autoimmune cytosolic pattern recognition receptors nucleic acid sensors interferon |
| title | Making Sense of Intracellular Nucleic Acid Sensing in Type I Interferon Activation in Sjögren’s Syndrome |
| title_full | Making Sense of Intracellular Nucleic Acid Sensing in Type I Interferon Activation in Sjögren’s Syndrome |
| title_fullStr | Making Sense of Intracellular Nucleic Acid Sensing in Type I Interferon Activation in Sjögren’s Syndrome |
| title_full_unstemmed | Making Sense of Intracellular Nucleic Acid Sensing in Type I Interferon Activation in Sjögren’s Syndrome |
| title_short | Making Sense of Intracellular Nucleic Acid Sensing in Type I Interferon Activation in Sjögren’s Syndrome |
| title_sort | making sense of intracellular nucleic acid sensing in type i interferon activation in sjogren s syndrome |
| topic | Sjögren’s syndrome autoimmune cytosolic pattern recognition receptors nucleic acid sensors interferon |
| url | https://www.mdpi.com/2077-0383/10/3/532 |
| work_keys_str_mv | AT erikahuijser makingsenseofintracellularnucleicacidsensingintypeiinterferonactivationinsjogrenssyndrome AT marjanaversnel makingsenseofintracellularnucleicacidsensingintypeiinterferonactivationinsjogrenssyndrome |