ReviewFrontiers in microbiology2016
Update on Pyrin Functions and Mechanisms of Familial Mediterranean Fever.
Review in Frontiers in microbiology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 47 papers, 1 of them a synthesis that pooled it.
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Who cites it
47 citing papers in PubMed, 1 synthesis or guideline pooled it, 89 citations in OpenAlex.
- To TRIM the Immunity: From Innate to Adaptive Immunity.Frontiers in immunology · 2020Pooled it
- Effectiveness of colchicine preparation switching in pediatric colchicine-resistant familial Mediterranean fever: a real-world study.European journal of pediatrics · 2026Article
- Article
- MEFV variant spectrum and exploratory in silico stratification of uncommon variants in 611 individuals tested for suspected familial Mediterranean fever in the Eastern Black Sea region of Türkiye.BMC genomic data · 2026Article
- TRIM proteins as emerging regulators of immune pathways: potential therapeutic targets in immune-related disorders.Frontiers in immunology · 2026Review
- miR-451a and IL18 can differentiate familial Mediterranean fever patients in attack and remission periods: a prospective cross-sectional study.Clinical rheumatology · 2025Article
- Arterial Stiffness and Subclinical Inflammation in Children with Familial Mediterranean Fever: A Comprehensive Analysis.Children (Basel, Switzerland) · 2025Article
- Association of Inflammasome Gene Expression Levels with Pathogenesis of Familial Mediterranean Fever in Armenians.International journal of molecular sciences · 2024Article
- A plasmonic biosensor pre-diagnostic tool for Familial Mediterranean Fever.Nature communications · 2024Article
- Altered expression of miR-17 and miR-148b in pediatric familial mediterranean fever patients.Clinical rheumatology · 2024Article
- Factors triggering familial mediterranean fever attacks, do they really exist?Internal and emergency medicine · 2024Article
- Very-early-onset Inflammatory Bowel Disease in an Infant with a Partial RIPK1 Deletion.Journal of clinical immunology · 2024Article
- Multipronged regulation of autophagy and apoptosis: emerging role of TRIM proteins.Cellular & molecular biology letters · 2024Review
- Phenotype-genotype correlation in children with familial Mediterranean fever in Morocco.Qatar medical journal · 2024Article
- Genewise detection of variants inFrontiers in genetics · 2024Article
- Concurrence of familial Mediterranean fever and Behçet's disease: a case report and review of the literature.Journal of medical case reports · 2023Review
- Genetic and Epigenetic Regulation of MEFV Gene and Their Impact on Clinical Outcome in Auto-Inflammatory Familial Mediterranean Fever Patients.Current issues in molecular biology · 2023Article
- A detailed molecular network map and model of the NLRP3 inflammasome.Frontiers in immunology · 2023Review
- Immunogenic Potential of the Mediterranean Fever Gene in Patients with Coronavirus Disease: A Cross-Sectional Study.Iranian journal of medical sciences · 2023Article
- Functional Assessment of Disease-Associated Pyrin Variants.Methods in molecular biology (Clifton, N.J.) · 2022Article
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mutations in the MEFV gene, which encodes the protein named pyrin (also called marenostrin or TRIM20), are associated with the autoinflammatory disease familial Mediterranean fever (FMF). Recent genetic and immunologic studies uncovered novel functions of pyrin and raised several new questions in relation to FMF pathogenesis. The disease is clinically heterogeneous reflecting the complexity and multiplicity of pyrin functions. The main functions uncovered so far include its involvement in innate immune response such as the inflammasome assemblage and, as a part of the inflammasome, sensing intracellular danger signals, activation of mediators of inflammation, and resolution of inflammation by the autophagy of regulators of innate immunity. Based on these functions, the FMF-associated versions of pyrin confer a heightened sensitivity to a variety of intracellular danger signals and postpone the resolution of innate immune responses. It remains to be demonstrated, however, what kind of selective advantage the heterozygous carriage conferred in the past to be positively selected and maintained in populations from the Mediterranean basin.
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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.