The pyrin inflammasome, a leading actor in pediatric autoinflammatory diseases

• DOI: 10.3389/fimmu.2023.1341680
• Publication Date: 2024-01-05T05:02:04Z
• Authors: Saverio La Bella; Armando Di Ludovico; Giulia Di Donato; Ozge Basaran; Seza Ozen; Marco Gattorno; Francesco Chiarelli; Luciana Breda
• Keywords: Pyrin domain; MEFV; Pyroptosis

The activation of the pyrin inflammasome represents a highly intriguing mechanism employed by innate immune system to effectively counteract pathogenic agents. Despite its key role in immunity, has also garnered significant attention due association with range autoinflammatory diseases (AIDs) including familial Mediterranean fever caused disruption MEFV gene, or other genes involved complex regulation mechanisms. Pyrin is strictly dependent on homeostasis-altering molecular processes, mostly consisting small Ras Homolog Family Member A (RhoA) GTPases pathogen toxins. downstream pathways are regulated phosphorylation specific residues kinases PKN1/2 and binding chaperone 14-3-3. Furthermore, played cytoskeleton gasdermin D, which responsible for membrane pores context pyroptosis. In addition, recent evidence highlighted steroid hormone catabolites alarmins S100A8/A9 S100A12 pyrin-dependent inflammation. aim this article offer comprehensive overview most better understand pathogenesis behind group pyrin-related AIDs.