Publication
Title
Recent insights in Pyrin inflammasome activation : identifying potential novel therapeutic approaches in Pyrin-associated autoinflammatory syndromes
Author
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.
Language
English
Source (journal)
Journal of clinical immunology. - New York, N.Y., 1981, currens
Publication
New york : Springer/plenum publishers , 2024
ISSN
0271-9142 [print]
1573-2592 [online]
DOI
10.1007/S10875-023-01621-5
Volume/pages
44 :1 (2024) , p. 1-13
Article Reference
8
ISI
001127428300011
Pubmed ID
38129719
Full text (Publisher's DOI)
Full text (open access)
The author-created version that incorporates referee comments and is the accepted for publication version Available from 22.06.2024
Full text (publisher's version - intranet only)
UAntwerpen
Faculty/Department
Research group
Publication type
Subject
Affiliation
Publications with a UAntwerp address
External links
Web of Science
Record
Identifier
Creation 01.02.2024
Last edited 08.02.2024
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