Results 51 to 60 of about 35,163 (154)
Molecular and Cellular Pediatrics, 2023 Background Familial Mediterranean fever (FMF) is a prototypical autoinflammatory syndrome associated with phagocytic cell activation. Pyrin mutations are the genetic basis of this disease, and its expression has been shown in monocytes, granulocytes ...
G. Varga +11 more
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Pyrin/marenostrin mutations in familial Mediterranean fever [PDF]
QJM, 1998 Familial Mediterranean fever (FMF) is an inherited inflammatory disease that is frequently complicated by reactive systemic (AA) amyloidosis. It is principally recognized in certain Mediterranean populations, and the diagnosis depends on clinical features.
D R, Booth +4 more
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PLoS ONE, 2018 Although the study of pathogen sensing by host defense systems continues to uncover a role for inflammasome components specific to particular pathogens, gaps remain in our knowledge.
Srabani Mitra +4 more
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Structural and Functional Analysis of the NLRP4 Pyrin Domain
Biochemistry, 2012 NLRP4 is a member of the nucleotide-binding and leucine-rich repeat receptor (NLR) family of cytosolic receptors and a member of an inflammation signaling cascade. Here, we present the crystal structure of the NLRP4 pyrin domain (PYD) at 2.3 Å resolution.
Eibl, Clarissa +11 more
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The NLRP12 Pyrin Domain: Structure, Dynamics, and Functional Insights [PDF]
Journal of Molecular Biology, 2011 The initial line of defense against infection is sustained by the innate immune system. Together, membrane-bound Toll-like receptors and cytosolic nucleotide-binding domain and leucine-rich repeat-containing receptors (NLR) play key roles in the innate immune response by detecting bacterial and viral invaders as well as endogenous stress signals.
Anderson S, Pinheiro +4 more
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International Journal of Immunopathology and Pharmacology, 2022 Introduction Dialysis-related amyloidosis (DRA) caused by β2-microgloblin (B2M) fibrils is a serious complication for patients with kidney failure on long-term dialysis.
Naoe Kaneko +5 more
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Physiological and pathophysiological functions of NLRP6: pro- and anti-inflammatory roles
Communications Biology, 2022 This review discusses emerging roles for the NLR family pyrin domain containing 6 receptor (NLRP6) in intestinal homeostasis, inflammation, cancer, the nervous system and liver disease.
Diego Angosto-Bazarra +2 more
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Identification of pyrin targets by CHiP-Seq [PDF]
Pediatric Rheumatology, 2015 Familial Mediterranean fever (FMF) is a recessive disorder characterized by episodes of fever and neutrophil-mediated 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.
Wood, G +5 more
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Dynamic disequilibrium-based pathogenicity model in mutated pyrin’s B30.2 domain—Casp1/p20 complex
Journal of Genetic Engineering and Biotechnology, 2022 Background The B30.2 variants lead to most relevant severity forms of familial Mediterranean fever (FMF) manifestations. The B30.2 domain plays a key role in protein-protein interaction (PPI) of pyrin with other apoptosis proteins and in regulation the ...
Alaaeldin G. Fayez +10 more
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TIR, CARD and PYRIN: three domains for an antimicrobial triad [PDF]
Cell Death & Differentiation, 2006 Innate immunity to microorganisms in mammals has gained a substantial interest during the last decade. The discovery of the Toll-like receptor (TLR) family has allowed the identification of a class of membrane-spanning receptors dedicated to microbial sensing. TLRs transduce downstream signaling via their intracellular Toll-interleukin-1 receptor (TIR)
C, Werts, S E, Girardin, D J, Philpott
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