The PYRIN Connection
نویسندگان
چکیده
Periodic fever syndromes (PFSs) comprise a subset of the hereditary autoinflammatory disorders that are defined by recurrent self-resolving attacks of systemic inflammatory reactions in the absence of infection or autoimmunity. Recent advances have led to the discovery that members of a new family of genes, the PYRIN family, account for several hereditary PFSs. Here we discuss new insights into the function of PYRIN proteins and the molecular basis of PFSs.
منابع مشابه
The PYRIN Connection: Novel Players in Innate Immunity and Inflammation
Periodic fever syndromes (PFSs) comprise a subset of the hereditary autoinflammatory disorders that are defined by recurrent self-resolving attacks of systemic inflammatory reactions in the absence of infection or autoimmunity. Recent advances have led to the discovery that members of a new family of genes, the PYRIN family, account for several hereditary PFSs. Here we discuss new insights into...
متن کاملThe death-domain fold of the ASC PYRIN domain, presenting a basis for PYRIN/PYRIN recognition.
The PYRIN domain is a conserved sequence motif identified in more than 20 human proteins with putative functions in apoptotic and inflammatory signalling pathways. The three-dimensional structure of the PYRIN domain from human ASC was determined by NMR spectroscopy. The structure determination reveals close structural similarity to death domains, death effector domains, and caspase activation a...
متن کاملPyrin levels in human monocytes and monocyte-derived macrophages regulate IL-1beta processing and release.
Macrophages and their precursors, monocytes, are key cells involved in the innate immune response. Although both monocytes and macrophages produce caspase-1, the key enzyme responsible for pro-IL-1beta processing; macrophages are limited in their ability to activate the enzyme and release functional IL-1beta. In this context, because mutations in the pyrin gene (MEFV) cause the inflammatory dis...
متن کاملSite-specific phosphorylation and microtubule dynamics control Pyrin inflammasome activation.
Pyrin, encoded by the MEFV gene, is best known for its gain-of-function mutations causing familial Mediterranean fever (FMF), an autoinflammatory disease. Pyrin forms a caspase-1-activating inflammasome in response to inactivating modifications of Rho GTPases by various bacterial toxins or effectors. Pyrin-mediated innate immunity is unique in that it senses bacterial virulence rather than micr...
متن کاملIdentification of pyrin targets by CHiP-Seq
Introduction Familial Mediterranean fever (FMF) is a recessive disorder characterized by episodes of fever and neutrophilmediated serosal inflammation. The gene causing FMF, MEFV, encodes a protein, pyrin. Pyrin is expressed predominantly in innate immune cells such as neutrophils, monocytes, and dendritic cells, but not in lymphocytes. Studies of pyrin localization show a cell-type dependency....
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عنوان ژورنال:
- The Journal of Experimental Medicine
دوره 200 شماره
صفحات -
تاریخ انتشار 2004