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http://hdl.handle.net/1942/42216
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DC Field | Value | Language |
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dc.contributor.author | WOUTERS, Flore | - |
dc.contributor.author | BOGIE, Jeroen | - |
dc.contributor.author | Wullaert, Andy | - |
dc.contributor.author | VAN DER HILST, Jeroen | - |
dc.date.accessioned | 2024-01-23T08:41:59Z | - |
dc.date.available | 2024-01-23T08:41:59Z | - |
dc.date.issued | 2024 | - |
dc.date.submitted | 2024-01-23T07:38:25Z | - |
dc.identifier.citation | JOURNAL OF CLINICAL IMMUNOLOGY, 44 (1) (Art N° 8) | - |
dc.identifier.uri | http://hdl.handle.net/1942/42216 | - |
dc.description.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. | - |
dc.language.iso | en | - |
dc.publisher | SPRINGER/PLENUM PUBLISHERS | - |
dc.rights | The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2023 | - |
dc.subject.other | Autoinflammatory syndromes | - |
dc.subject.other | FMF | - |
dc.subject.other | Pyrin | - |
dc.subject.other | inflammasome | - |
dc.subject.other | pharmacological therapy | - |
dc.title | Recent Insights in Pyrin Inflammasome Activation: Identifying Potential Novel Therapeutic Approaches in Pyrin-Associated Autoinflammatory Syndromes | - |
dc.type | Journal Contribution | - |
dc.identifier.issue | 1 | - |
dc.identifier.volume | 44 | - |
local.format.pages | 13 | - |
local.bibliographicCitation.jcat | A1 | - |
dc.description.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. | - |
dc.description.notes | flore.wouters@uhasselt.be; jeroen.vanderhilst@uhasselt.be | - |
local.publisher.place | 233 SPRING ST, NEW YORK, NY 10013 USA | - |
local.type.refereed | Refereed | - |
local.type.specified | Review | - |
local.bibliographicCitation.artnr | 8 | - |
dc.identifier.doi | 10.1007/s10875-023-01621-5 | - |
dc.identifier.pmid | 38129719 | - |
dc.identifier.isi | 001127428300011 | - |
dc.contributor.orcid | van der Hilst, Jeroen/0000-0003-4527-6310 | - |
local.provider.type | wosris | - |
local.description.affiliation | [Wouters, Flore; Bogie, Jeroen; van der Hilst, Jeroen] Hasselt Univ, Biomed Res Inst, Dept Immunol & Infect, B-3590 Diepenbeek, Belgium. | - |
local.description.affiliation | [Bogie, Jeroen] Univ MS Ctr Hasselt, B-3900 Pelt, Belgium. | - |
local.description.affiliation | [Wullaert, Andy] Univ Ghent, Dept Internal Med & Paediat, B-9000 Ghent, Belgium. | - |
local.description.affiliation | [Wullaert, Andy] VIB, VIB UGent Ctr Inflammat Res, B-9052 Ghent, Belgium. | - |
local.description.affiliation | [Wullaert, Andy] Univ Antwerp, Dept Biomed Sci, Lab Prot Sci Prote & Epigenet Signalling PPES, Antwerp, Belgium. | - |
local.description.affiliation | [van der Hilst, Jeroen] Jessa Gen Hosp, Dept Infect Dis & Immune Pathol, Hasselt, Belgium. | - |
local.description.affiliation | [van der Hilst, Jeroen] Limburg Clin Res Ctr, Hasselt, Belgium. | - |
local.uhasselt.international | no | - |
item.accessRights | Embargoed Access | - |
item.fullcitation | WOUTERS, Flore; BOGIE, Jeroen; Wullaert, Andy & VAN DER HILST, Jeroen (2024) Recent Insights in Pyrin Inflammasome Activation: Identifying Potential Novel Therapeutic Approaches in Pyrin-Associated Autoinflammatory Syndromes. In: JOURNAL OF CLINICAL IMMUNOLOGY, 44 (1) (Art N° 8). | - |
item.fulltext | With Fulltext | - |
item.embargoEndDate | 2024-06-22 | - |
item.contributor | WOUTERS, Flore | - |
item.contributor | BOGIE, Jeroen | - |
item.contributor | Wullaert, Andy | - |
item.contributor | VAN DER HILST, Jeroen | - |
crisitem.journal.issn | 0271-9142 | - |
crisitem.journal.eissn | 1573-2592 | - |
Appears in Collections: | Research publications |
Files in This Item:
File | Description | Size | Format | |
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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 Until 2024-06-22 | Peer-reviewed author version | 3.97 MB | Adobe PDF | View/Open Request a copy |
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