Introduction MEFV which encodes pyrin, cause familial Mediterranean fever (FMF), the most common auto-inflammatory disease. The pyrin protein appears to be a regulator of inflammation, but its exact role on inflammatory pathways is still controversial. Several pyrin-interacting proteins have been identified, each of which are related to inflammation through regulation of cell death, cytokine se...

Introduction The gene causing familial Mediterranean fever (FMF), MEFV, encodes a protein, pyrin, which is expressed at high levels in granulocytes, monocytes, dendritic cells and in some human myeloid leukemia cell lines, such as THP.1. Studies of pyrin localization show a cell-type dependency. In transfection experiments full-length pyrin is cytoplasmic and associates with the cytoskeleton. H...

Pyrin, the familial Mediterranean fever protein, is found in association with the cytoskeleton in myeloid/monocytic cells and modulates IL-1beta processing, NF-kappaB activation, and apoptosis. These effects are mediated in part through cognate interactions with the adaptor protein ASC, which shares an N-terminal motif with pyrin. We sought additional upstream regulators of inflammation by usin...

PSTPIP1 is a cytoskeleton-associated adaptor protein that links PEST-type phosphatases to their substrates. Mutations in PSTPIP1 cause PAPA syndrome (Pyogenic sterile Arthritis, Pyoderma gangrenosum, and Acne), an autoinflammatory disease. PSTPIP1 binds to pyrin and mutations in pyrin result in familial Mediterranean fever (FMF), a related autoinflammatory disorder. Since disease-associated mut...

Introduction Familial Mediterranean fever (FMF) is one of the most common autoinflammatory disorders and is characterized by episodic attacks of fever, along with inflammation. FMF pathogenesis is associated with various mutations in the MEFV gene, which encodes pyrin. Pyrin is expressed predominantly in neutrophils that have an important role in the innate immune response. Several proteins rel...

Hemorrhagic shock (HS) promotes the development of systemic inflammatory response syndrome and organ injury by activating and priming the innate immune system for an exaggerated inflammatory response through, as of yet, unclear mechanisms. IL-1β also plays an important role in the development of post-HS systemic inflammatory response syndrome and active IL-1β production is tightly controlled by...

BACKGROUND Familial Mediterranean fever (FMF) is a recessively inherited disease with a variety of clinical presentations. The disease is associated with mutations in the FMF gene (MEFV), which encodes for the pyrin protein. The role of the E148Q pyrin mutation in the FMF phenotype remains inconclusive, and some authors even view it as a disease-insignificant polymorphism. The calculated change...

Familial Mediterranean fever (FMF) is a recessive disorder characterized by episodes of fever and intense inflammation. FMF attacks are unique in their sensitivity to the microtubule inhibitor colchicine, contrasted with their refractoriness to the anti-inflammatory effects of glucocorticoids. The FMF gene, MEFV, was recently identified by positional cloning; it is expressed at high levels in g...