Introduction The familial Mediterranean fever (FMF, OMIM 249100) is an autosomal recessive auto-inflammatory disease primarily occuring in Armenian, Turkish, Jewish and Arabic populations. The first clinical symptoms of FMF usually appear in childhood. The chronic relapsing inflammation of the serous membranes leads to febrile attacks often associated with abdominal, joint and/or chest pains. T...

A series of benzo[d]imidazole analogues of thiabenzole were synthesized and their antiinflammatory activities toward NLRP3 (nucleotide-binding domain leucine-rich repeat containing protein family，pyrin domain-containing 3，also known as cryopyrin or NALP3) inflammasome were evaluated in vitro. Two lead compounds, TBZ-09 and TBZ-21, were identified by antiproduction of IL-1β. In the second round ...

Introduction Familial Mediterranean Fever (FMF) is caused by mutations in the MEFV (Mediterranean FeVer) gene, which encodes pyrin. Phenotypic heterogeneity is very common in FMF patients and may partly rely on genetic heterogeneity. However, many cases having weak phenotypic-genotypic correlation, different clinical findings and therapeutic approaches with the same genotype show that FMF is no...

BACKGROUND Cryopyrin-associated periodic syndrome (CAPS) is a group of rare, heterogeneous autoinflammatory disease characterized by interleukin (IL)-1β-mediated systemic inflammation and clinical symptoms involving skin, joints, central nervous system, and eyes. It encompasses a spectrum of three clinically overlapping autoinflammatory syndromes including familial cold autoinflammatory syndrom...

Pathogen recognition by intracellular sensors involves the assembly of a caspase-1 activation machine termed the inflammasome. Intracellular pathogens like Francisella that gain access to the cytosolic detection systems are useful tools to uncover the details of caspase-1 activation events. This review overviews Francisella function in the mononuclear phagocyte with particular attention to infl...