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

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. Access through your institution

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BEING VIEWED BY OTHERS MUTATIONS IN THE B30.2 AND THE CENTRAL HELICAL SCAFFOLD DOMAINS OF PYRIN DIFFERENTIALLY AFFECT INFLAMMASOME ACTIVATION Article Open access 25 March 2023 FAMILIAL

MEDITERRANEAN FEVER-RELATED MIR-197-3P TARGETS _IL1R1_ GENE AND MODULATES INFLAMMATION IN MONOCYTES AND SYNOVIAL FIBROBLASTS Article Open access 12 January 2021 THE PAST 25 YEARS IN

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CHRU of Montpellier (PHRC2005), the National Human Genome Research Institute, INSERM and Istanbul Faculty of Medicine. We thank the clinicians for providing patient samples, J Tazy for

helpful discussion and M Vittal for English editing of the manuscript. AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Unité médicale des maladies auto-inflammatoires, CHRU de Montpellier,

INSERM U844, UM1, Hopital Arnaud de Villeneuve, Montpellier, France S Grandemange & I Touitou * National Human Genome Research Institute, Bethesda, MD, USA I Aksentijevich * INSERM U933,

Université Pierre et Marie Curie-Paris6 UMR S933, Paris, France I Jeru * Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University,

Istanbul, Turkey A Gul Authors * S Grandemange View author publications You can also search for this author inPubMed Google Scholar * I Aksentijevich View author publications You can also

search for this author inPubMed Google Scholar * I Jeru View author publications You can also search for this author inPubMed Google Scholar * A Gul View author publications You can also

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Touitou. ETHICS DECLARATIONS COMPETING INTERESTS The authors declare no conflict of interest RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Grandemange,

S., Aksentijevich, I., Jeru, I. _et al._ The regulation of MEFV expression and its role in health and familial Mediterranean fever. _Genes Immun_ 12, 497–503 (2011).

https://doi.org/10.1038/gene.2011.53 Download citation * Received: 20 May 2011 * Accepted: 27 June 2011 * Published: 21 July 2011 * Issue Date: October 2011 * DOI:

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currently available for this article. Copy to clipboard Provided by the Springer Nature SharedIt content-sharing initiative KEYWORDS * expression * _MEFV_ * FMF