We have identified a novel gene encoding a pyrin domain protein of 89 amino acids that is expressed in various tissues including liver, brain, and spleen. The protein is highly homologous to the pyrin domain of apoptosis-associated speck-like protein (ASC). Therefore, we termed it ASC-like (ASCL). We found that ASCL gene was densely and frequently (80%) methylated in hepatocellular carcinoma (H...

The human interferon-inducible protein IFI16 is an important antiviral factor that binds nuclear viral DNA and promotes antiviral responses. Here, we define IFI16 dynamics in space and time and its distinct functions from the DNA sensor cyclic dinucleotide GMP-AMP synthase (cGAS). Live-cell imaging reveals a multiphasic IFI16 redistribution, first to viral entry sites at the nuclear periphery a...

Objective Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of fever and peritonitis, pleuritis, arthritis or an erysipelaslike skin disorder. The disease may present at any age, more than 80% of patients being symptomatic by the age of 20 yr. Its main long-term complication is amyloid A (AA) amyloidosis, a severe manifestation with poor pro...

Introduction Familial Mediterranean fever (FMF) is an autoinflammatory disease which is characterized by recurrent episodes of typical attacks with fever and abdominal pain. The pathophysiology shows an ineffective pyrin which leads to brakeless inflammation. It is not exactly known which factors lead to initiation of the attacks. Furthermore there seems to be sustained inflammation between the...

Nucleotide-binding and oligomerization domain (NOD)-like receptors (NLRs) play important role in immune responses by regulating nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB), type I interferon and inflammasomes. Nod-like receptor pyrin domain-containing protein 6 (NLRP6) is vital for protection of mice from colitis and colorectal tumorigenesis. However, the role of NLRP...

Introduction Familial Mediterranean fever (FMF) is the most common of the hereditary autoinflammatory disorders. FMF is caused by mutations of MEFV gene which encodes for pyrin. It has been recently reported that frequency of FMF-like symptoms decreases from patients carrying two high penetrance mutations towards patients with a single low penetrance mutation. The effectiveness of interleukin (...

Background Uncontrollable neutrophil activation and its migration to serous tissues that result in oxidative stress responsible for the tissue damage due to pyrin mutation in FMF patients. Reactive oxygen radicals are released by active neutrophils and the basic cell compounds such as nucleic acids, proteins and lipids during the inflammation. The aim of our study was to measure plasma advanced...