ReviewGenes and immunity2011
The regulation of MEFV expression and its role in health and familial Mediterranean fever.
Review in Genes and immunity, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed.
- Pre-electroacupuncture Ameliorates Cerebral Ischemia-reperfusion Injury by Inhibiting Microglial RhoA/pyrin/GSDMD Signaling Pathway.Neurochemical research · 2024Article
- Implementation of long-read sequencing for routine molecular diagnosis of familial mediterranean fever.Practical laboratory medicine · 2024Article
- Anxiety and depression among patients with familial Mediterranean fever.Zeitschrift fur Rheumatologie · 2024Article
- Galantamine attenuates autoinflammation in a mouse model of familial mediterranean fever.Molecular medicine (Cambridge, Mass.) · 2022Article
- Article
- Fear Stress During Pregnancy Affects Placental m6A-Modifying Enzyme Expression and Epigenetic Modification Levels.Frontiers in genetics · 2022Article
- The Persistent Pain Transcriptome: Identification of Cells and Molecules Activated by Hyperalgesia.The journal of pain · 2021Article
- Transcriptional Regulation of Inflammasomes.International journal of molecular sciences · 2020Review
- ProbioticFrontiers in immunology · 2018Article
- Carbon monoxide decreases interleukin-1β levels in the lung through the induction of pyrin.Cellular & molecular immunology · 2017Article
- Increased prevalence of MEFV exon 10 variants in Japanese patients with adult-onset Still's disease.Clinical and experimental immunology · 2015Article
- Ron receptor-dependent gene regulation of Kupffer cells during endotoxemia.Hepatobiliary & pancreatic diseases international : HBPD INT · 2014Article
- Hemorrhagic shock augments Nlrp3 inflammasome activation in the lung through impaired pyrin induction.Journal of immunology (Baltimore, Md. : 1950) · 2013Article
- Monogenic autoinflammatory syndromes: state of the art on genetic, clinical, and therapeutic issues.International journal of rheumatology · 2013Review
- Increased levels of macrophage migration inhibitory factor in patients with familial mediterranean Fever.International journal of medical sciences · 2013Article
- Genetic loss of murine pyrin, the Familial Mediterranean Fever protein, increases interleukin-1β levels.PloS one · 2012Article
Corrections and comments
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Authors and funding
5 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Familial Mediterranean fever (FMF) is a hereditary recurrent fever associated with mutations in the gene MEFV encoding pyrin. It is expressed mainly in neutrophils and macrophages, and modulates the production of the potent pro-inflammatory cytokine interleukin-1β through regulation of nuclear factor-κB and caspase-1. The MEFV gene expression depends on multiple levels of regulation. Sequence variants located in the promoter and at the 3'-untranslated region of the gene modulate this expression. Two studies demonstrated decreased mRNA levels in FMF patients compared with healthy subjects, whereas two others found no significant differences. The diverse experimental settings may have resulted in variable quantification of the 15 splice variants that have been identified recently. Some of these isoforms are regulated by nonsense-mediated decay in both cell- and transcript-specific manner, and may be differentially translated in THP1 cells. In addition, pyrin may be cleaved by caspase 1. The full-length pyrin was less abundant than the cleaved fragment in mononuclear cells from FMF patients than in controls, whereas the opposite was observed in granulocytes. Altogether, the regulation of MEFV expression is more complex than anticipated in both physiological and pathological conditions. Its deregulation is likely to alter the inflammasome function and subsequently result in uncontrolled inflammation as seen in FMF.
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