Recent Insights in Pyrin inflammasome activation : identifying potential novel therapeutic approaches in Pyrin-associated autoinflammatory syndromes
- Author
- Flore Wouters, Jeroen Bogie, Andy Wullaert (UGent) and Jeroen van der Hilst
- Organization
- 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.
- Keywords
- pharmacological therapy, inflammasome, Pyrin, FMF, Autoinflammatory syndromes
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Citation
Please use this url to cite or link to this publication: http://hdl.handle.net/1854/LU-01HMBC74Q26R1KTTG4G308SSMB
- MLA
- Wouters, Flore, et al. “Recent Insights in Pyrin Inflammasome Activation : Identifying Potential Novel Therapeutic Approaches in Pyrin-Associated Autoinflammatory Syndromes.” JOURNAL OF CLINICAL IMMUNOLOGY, vol. 44, no. 1, 2024, doi:10.1007/s10875-023-01621-5.
- APA
- Wouters, F., Bogie, J., Wullaert, A., & van der Hilst, J. (2024). Recent Insights in Pyrin inflammasome activation : identifying potential novel therapeutic approaches in Pyrin-associated autoinflammatory syndromes. JOURNAL OF CLINICAL IMMUNOLOGY, 44(1). https://doi.org/10.1007/s10875-023-01621-5
- Chicago author-date
- Wouters, Flore, Jeroen Bogie, Andy Wullaert, and Jeroen van der Hilst. 2024. “Recent Insights in Pyrin Inflammasome Activation : Identifying Potential Novel Therapeutic Approaches in Pyrin-Associated Autoinflammatory Syndromes.” JOURNAL OF CLINICAL IMMUNOLOGY 44 (1). https://doi.org/10.1007/s10875-023-01621-5.
- Chicago author-date (all authors)
- Wouters, Flore, Jeroen Bogie, Andy Wullaert, and Jeroen van der Hilst. 2024. “Recent Insights in Pyrin Inflammasome Activation : Identifying Potential Novel Therapeutic Approaches in Pyrin-Associated Autoinflammatory Syndromes.” JOURNAL OF CLINICAL IMMUNOLOGY 44 (1). doi:10.1007/s10875-023-01621-5.
- Vancouver
- 1.Wouters F, Bogie J, Wullaert A, van der Hilst J. Recent Insights in Pyrin inflammasome activation : identifying potential novel therapeutic approaches in Pyrin-associated autoinflammatory syndromes. JOURNAL OF CLINICAL IMMUNOLOGY. 2024;44(1).
- IEEE
- [1]F. Wouters, J. Bogie, A. Wullaert, and J. van der Hilst, “Recent Insights in Pyrin inflammasome activation : identifying potential novel therapeutic approaches in Pyrin-associated autoinflammatory syndromes,” JOURNAL OF CLINICAL IMMUNOLOGY, vol. 44, no. 1, 2024.
@article{01HMBC74Q26R1KTTG4G308SSMB,
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.}},
articleno = {{8}},
author = {{Wouters, Flore and Bogie, Jeroen and Wullaert, Andy and van der Hilst, Jeroen}},
issn = {{0271-9142}},
journal = {{JOURNAL OF CLINICAL IMMUNOLOGY}},
keywords = {{pharmacological therapy,inflammasome,Pyrin,FMF,Autoinflammatory syndromes}},
language = {{eng}},
number = {{1}},
pages = {{13}},
title = {{Recent Insights in Pyrin inflammasome activation : identifying potential novel therapeutic approaches in Pyrin-associated autoinflammatory syndromes}},
url = {{http://doi.org/10.1007/s10875-023-01621-5}},
volume = {{44}},
year = {{2024}},
}
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