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Title
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Recent insights in Pyrin inflammasome activation : identifying potential novel therapeutic approaches in Pyrin-associated autoinflammatory syndromes
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Author
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Abstract
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| 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. |
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Language
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English
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Source (journal)
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Journal of clinical immunology. - New York, N.Y., 1981, currens
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Publication
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New york
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Springer/plenum publishers
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2024
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ISSN
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0271-9142
[print]
1573-2592
[online]
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DOI
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10.1007/S10875-023-01621-5
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Volume/pages
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44
:1
(2024)
, p. 1-13
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Article Reference
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8
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ISI
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001127428300011
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Pubmed ID
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38129719
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Full text (Publisher's DOI)
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Full text (open access)
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Full text (publisher's version - intranet only)
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