Dysregulation of innate immunity: hereditary periodic fever syndromes
نویسندگان
چکیده
منابع مشابه
Hereditary periodic fever syndromes.
The hereditary periodic fevers are a group of Mendelian disorders characterized by seemingly unprovoked fever and localized inflammation. Recent data indicate that these illnesses represent inborn errors in the regulation of innate immunity. Pyrin, the protein mutated in familial Mediterranean fever, defines an N-terminal domain found in a large family of proteins involved in inflammation and a...
متن کاملHereditary periodic fever syndromes
osting by E Abstract Hereditary periodic fever syndromes, comprise a group of hereditary disorders with similar clinical features of recurrent short episodes of fever associated with inflammatory manifestations. These are usually self-limited in nature and occur in the absence of infection or autoimmune reaction. Between attacks, patients feel well and regain their normal daily functions until ...
متن کاملAutoinflammatory diseases: the hereditary periodic fever syndromes.
Human autoinflammatory diseases (HAIDs) are a heterogeneous group of genetically determined affections characterized by seemingly unprovoked inflammation, in the absence of autoimmune or infective causes. The hereditary periodic fever syndromes (HPFSs) are a HAID subset consisting of three main nosologic entities: familial Mediterranean fever (FMF), hyperimmunoglobulinemia D and periodic fever ...
متن کاملPathogenesis of familial periodic fever syndromes or hereditary autoinflammatory syndromes.
Familial periodic fever syndromes, otherwise known as hereditary autoinflammatory syndromes, are inherited disorders characterized by recurrent episodes of fever and inflammation. The general hypothesis is that the innate immune response in these patients is wrongly tuned, being either too sensitive to very minor stimuli or turned off too late. The genetic background of the major familial perio...
متن کاملMutations in NALP12 cause hereditary periodic fever syndromes.
NALP proteins, also known as NLRPs, belong to the CATERPILLER protein family involved, like Toll-like receptors, in the recognition of microbial molecules and the subsequent activation of inflammatory and immune responses. Current advances in the function of NALPs support the recently proposed model of a disease continuum bridging autoimmune and autoinflammatory disorders. Among these diseases,...
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ژورنال
عنوان ژورنال: British Journal of Haematology
سال: 2009
ISSN: 0007-1048,1365-2141
DOI: 10.1111/j.1365-2141.2008.07036.x