ArticleNature immunology2016
Pyrin inflammasome activation and RhoA signaling in the autoinflammatory diseases FMF and HIDS.
Article in Nature immunology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 302 papers, 2 of them syntheses that pooled it.
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Who cites it
302 citing papers in PubMed, 2 syntheses or guidelines pooled it, 553 citations in OpenAlex.
- Colchicine may become a new cornerstone therapy for coronary artery disease: a meta-analysis of randomized controlled trials.Clinical rheumatology · 2022Pooled it
- Interventions for reducing inflammation in familial Mediterranean fever.The Cochrane database of systematic reviews · 2022Pooled it
- Long-term efficacy and safety of canakinumab in patients with colchicine-resistant familial Mediterranean fever: results from the randomised phase III CLUSTER trial.Annals of the rheumatic diseases · 2020Trial
- A systems perspective on monoallelic inborn errors of immunity.Nature genetics · 2026Review
- WNK1 links ionic signals to microtubule dynamics to control pyrin inflammasome assembly.Science advances · 2026Article
- The gasdermin family: from pyroptosis mechanisms to therapeutic targets.Signal transduction and targeted therapy · 2026Review
- Familial Mediterranean fever in Azerbaijani children: a nationwide cohort study of genotype-phenotype association.Rheumatology (Oxford, England) · 2026Article
- Innate immune sensors of inflammasomes and PANoptosomes: structural-mechanistic insights and therapeutic implications.Cell research · 2026Review
- Arrhythmic Mitral Valve Prolapse as a Regional Stretch-Induced Cardiomyopathy with Fibro-Inflammatory Signature: A Conceptual Review.Journal of cardiovascular development and disease · 2026Review
- [Regulatory mechanisms of post-translational modifications in inflammasome signaling: a review].Zhejiang da xue xue bao. Yi xue ban = Journal of Zhejiang University. Medical sciences · 2026Review
- Decoding the shared genetic liability of lower respiratory tract infections via genomic structural equation modeling.BMC pulmonary medicine · 2026Article
- Clinical Association of Pan-Immune-Inflammation Value with MEFV Mutation Burden and Amyloidosis in Adults with Familial Mediterranean Fever: A Retrospective Cohort Study.Journal of clinical medicine · 2026Article
- ThebioRxiv : the preprint server for biology · 2026Article
- Structural basis of the protein kinase PKN1 HR1 domain oligomerization and differential regulation by RhoA and Rac1.The Journal of biological chemistry · 2026Article
- Nano-granulated zoledronate sensitizes innate immune metabolism to enhance vaccine-induced and antitumor immunity.Cell reports. Medicine · 2026Article
- AI platform for CRISPR functional mapping and function-based drug design.bioRxiv : the preprint server for biology · 2026Article
- Cofilin-1 is a redox-sensitive guard of the NLRP3 inflammasome.Nature immunology · 2026Article
- Mechanisms and therapeutic potential of colchicine in atherosclerotic cardiovascular disease.Nature cardiovascular research · 2026Review
- An overview of human inflammasomes: Activation and regulation.Journal of immunology (Baltimore, Md. : 1950) · 2026Review
- Monogenic disorders of immunity: Common variants are not so rare.Cell genomics · 2026Review
242 more citing papers are in PubMed but not listed here.
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Mutations in the genes encoding pyrin and mevalonate kinase (MVK) cause distinct interleukin-1β (IL-1β)-mediated autoinflammatory diseases: familial Mediterranean fever (FMF) and hyperimmunoglobulinemia D syndrome (HIDS). Pyrin forms an inflammasome when mutant or in response to bacterial modification of the GTPase RhoA. We found that RhoA activated the serine-threonine kinases PKN1 and PKN2 that bind and phosphorylate pyrin. Phosphorylated pyrin bound to 14-3-3 proteins, regulatory proteins that in turn blocked the pyrin inflammasome. The binding of 14-3-3 and PKN proteins to FMF-associated mutant pyrin was substantially decreased, and the constitutive IL-1β release from peripheral blood mononuclear cells of patients with FMF or HIDS was attenuated by activation of PKN1 and PKN2. Defects in prenylation, seen in HIDS, led to RhoA inactivation and consequent pyrin inflammasome activation. These data suggest a previously unsuspected fundamental molecular connection between two seemingly distinct autoinflammatory disorders.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.