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Title: | Recent Insights in Pyrin Inflammasome Activation: Identifying Potential Novel Therapeutic Approaches in Pyrin-Associated Autoinflammatory Syndromes | Authors: | WOUTERS, Flore BOGIE, Jeroen Wullaert, Andy VAN DER HILST, Jeroen |
Issue Date: | 2024 | Publisher: | SPRINGER/PLENUM PUBLISHERS | Source: | JOURNAL OF CLINICAL IMMUNOLOGY, 44 (1) (Art N° 8) | Abstract: | Pyrin is a cytosolic protein encoded by the MEFV gene, predominantly expressed in innate immune cells. Upon activation, it forms an inflammasome, a multimolecular complex that enables the activation and secretion of IL-1 beta and IL-18. In addition, the Pyrin inflammasome activates Gasdermin D leading to pyroptosis, a highly pro-inflammatory cell death. Four autoinflammatory syndromes are associated with Pyrin inflammasome dysregulation: familial Mediterranean fever, hyper IgD syndrome/mevalonate kinase deficiency, pyrin-associated autoinflammation with neutrophilic dermatosis, and pyogenic arthritis, pyoderma gangrenosum, and acne syndrome. In this review, we discuss recent advances in understanding the molecular mechanisms regulating the two-step model of Pyrin inflammasome activation. Based on these insights, we discuss current pharmacological options and identify a series of existing molecules with therapeutic potential for the treatment of pyrin-associated autoinflammatory syndromes. | Notes: | Wouters, F; van der Hilst, J (corresponding author), Hasselt Univ, Biomed Res Inst, Dept Immunol & Infect, B-3590 Diepenbeek, Belgium.; van der Hilst, J (corresponding author), Jessa Gen Hosp, Dept Infect Dis & Immune Pathol, Hasselt, Belgium.; van der Hilst, J (corresponding author), Limburg Clin Res Ctr, Hasselt, Belgium. flore.wouters@uhasselt.be; jeroen.vanderhilst@uhasselt.be |
Keywords: | Autoinflammatory syndromes;FMF;Pyrin;inflammasome;pharmacological therapy | Document URI: | http://hdl.handle.net/1942/42216 | ISSN: | 0271-9142 | e-ISSN: | 1573-2592 | DOI: | 10.1007/s10875-023-01621-5 | ISI #: | 001127428300011 | Rights: | The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2023 | Category: | A1 | Type: | Journal Contribution |
Appears in Collections: | Research publications |
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Recent Insights in Pyrin Inflammasome Activation_ Identifying Potential.pdf Restricted Access | Published version | 1.41 MB | Adobe PDF | View/Open Request a copy |
08dqKkwn-TP4.pdf | Peer-reviewed author version | 3.97 MB | Adobe PDF | View/Open |
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