Results 11 to 20 of about 44,417 (236)

A New Twist on the PYRIN Mediterranean Coast [PDF]

Immunity, 2011
Familial Mediterranean fever is caused by mutations of the PYRIN protein. Chae et al. (2011) provide evidence for a ASC protein-dependent pathway of caspase-1 activation in which gain-of-function PYRIN mutations lead to IL-1β cytokine overproduction and inflammatory disease.
Franchi, Luigi, Núñez, Gabriel
openaire   +3 more sources

Mutations in the B30.2 and the central helical scaffold domains of pyrin differentially affect inflammasome activation

Cell Death and Disease, 2023
Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disorder. FMF is caused by mutations in the MEFV gene, encoding pyrin, an inflammasome sensor.
Daria Chirita, Pauline Bronnec, Flora Magnotti, Sarah Dalmon, Amandine Martin, Michel Popoff, Mathieu Gerfaud-Valentin, Pascal Sève, Alexandre Belot, Anne Contis, Agnes Duquesne, Gaetane Nocturne, Irene Lemelle, Sophie Georgin-Lavialle, Guilaine Boursier, Isabelle Touitou, Yvan Jamilloux, Thomas Henry   +17 more
doaj   +2 more sources

Genetic Analysis of MEFV Gene Pyrin Domain in Patients With Behçet's Disease [PDF]

Mediators of Inflammation, 2006
Objectives. Behçet's disease (BD) is a systemic vasculitis with recurrent oral and genital ulcers and uveitis. MEFV gene, which is the main factor in familial Mediterranean fever (FMF), is also reported to be a susceptibility gene for BD.
Ahmet Dursun, Hatice Gul Durakbasi-Dursun, Ayse Gul Zamani, Zerrin Gülin Gulbahar, Recep Dursun, Cengiz Yakicier   +5 more
doaj   +3 more sources

Transcriptional licensing is required for Pyrin inflammasome activation in human macrophages and bypassed by mutations causing familial Mediterranean fever. [PDF]

PLoS Biology, 2022
Pyrin is a cytosolic immune sensor that nucleates an inflammasome in response to inhibition of RhoA by bacterial virulence factors, triggering the release of inflammatory cytokines, including IL-1β.
Matthew S J Mangan, Matthew S J Mangan, Friederike Gorki, Karoline Krause, Alexander Heinz, Anne Pankow, Thomas Ebert, Dieter Jahn, Karsten Hiller, Veit Hornung, Marcus Maurer, Florian I Schmidt, Ralf Gerhard, Eicke Latz   +13 more
doaj   +2 more sources

Kolşisinin pyrin ve pyrin ile ilişkili proteinler üzerine etkisi

, 2012
Taşkıran ZE. Kolşisinin Pyrin ve Pyrin ile İlişkili Proteinler Üzerine Etkisi. Hacettepe Üniversitesi, Sağlık Bilimleri Enstitüsü, Tıbbi Biyoloji Doktora Tezi, Ankara, 2012. Otoinflamatuvar hastalıklar içinde en yaygın olanı ailevi Akdeniz ateşidir (AAA).
Taşkıran, Zihni Ekim
openaire   +3 more sources

A semi‐automated ASC speck assay to evaluate pyrin inflammasome activation [PDF]

Clinical & Translational Immunology
Objective To develop a rapid functional assay to validate variants of uncertain significance (VUS) in the MEFV gene. Methods Overactivity of the pyrin inflammasome pathway and ASC speck oligomerisation in response to stimulation with low concentrations ...
Pei Dai, Oliver Skinner, Xufeng Lin, Aiden Telfser, Stephanie Ruiz‐Diaz, Rohit G Saldanha, Katie Frith, Ming‐Wei Lin, Kahn Preece, Paul E Gray, Alberto Pinzon‐Charry, Anna Sullivan, Stephen Adelstein, Winnie WY Tong, Matthew JS Parker, Laila Girgis, Brynn Wainstein, Samar Ojami, Elissa K Deenick, Leonard D Goldstein, Michael J Rogers, Tri Giang Phan   +21 more
doaj   +2 more sources

Pyrin inflammasome-driven erosive arthritis caused by unprenylated RHO GTPase signaling [PDF]

EMBO Molecular Medicine
Geranylgeranyl pyrophosphate, a non-sterol intermediate of the mevalonate pathway, serves as the substrate for protein geranylgeranylation, a process catalyzed by geranylgeranyl transferase I (GGTase-I).
Murali K Akula, Elisabeth Gilis, Pieter Hertens, Lieselotte Vande Walle, Mozes Sze, Julie Coudenys, Yunus Incik, Omar Khan, Martin O Bergo, Dirk Elewaut, Andy Wullaert, Mohamed Lamkanfi, Geert van Loo   +12 more
doaj   +2 more sources

Actin Remodeling Defects Leading to Autoinflammation and Immune Dysregulation

Frontiers in Immunology, 2021
A growing number of monogenic immune-mediated diseases have been related to genes involved in pathways of actin cytoskeleton remodeling. Increasing evidences associate cytoskeleton defects to autoinflammatory diseases and primary immunodeficiencies.
Riccardo Papa, Federica Penco, Stefano Volpi, Marco Gattorno   +3 more
doaj   +1 more source

Alpha-linolenic acid pretreatment alleviates NETs-induced alveolar macrophage pyroptosis by inhibiting pyrin inflammasome activation in a mouse model of sepsis-induced ALI/ARDS

Frontiers in Immunology, 2023
BackgroundNeutrophil extracellular traps (NETs) can cause acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) by inducing macrophage pyroptosis.
Chenchen Liu, Chenchen Liu, Yu Zhou, Qing Tu, Liangfang Yao, Jinbao Li, Zhongwei Yang   +6 more
doaj   +1 more source

Vasculitis and familial Mediterranean fever: Description of 22 French adults from the juvenile inflammatory rheumatism cohort

Frontiers in Medicine, 2022
ObjectiveThe frequency of vasculitis may be increased in patients with Familial Mediterranean Fever (FMF), according to several studies. Our aim was to assess the characteristics of French adult patients with both diseases.MethodsPatients with vasculitis
Salam Abbara, Jean-Benoit Monfort, Léa Savey, Philippe Moguelet, David Saadoun, Claude Bachmeyer, Olivier Fain, Benjamin Terrier, Zahir Amoura, Alexis Mathian, Laurent Gilardin, David Buob, Chantal Job-Deslandre, Jean-François Dufour, Rebecca Sberro-Soussan, Gilles Grateau, Sophie Georgin-Lavialle, Sophie Georgin-Lavialle   +17 more
doaj   +1 more source