Results 1 to 10 of about 35,163 (154)

The Pyrin Inflammasome in Health and Disease [PDF]

Frontiers in Immunology, 2019
The pyrin inflammasome has evolved as an innate immune sensor to detect bacterial toxin-induced Rho guanosine triphosphatase (Rho GTPase)-inactivation, a process that is similar to the “guard” mechanism in plants. Rho GTPases act as molecular switches to
Daniel Kästner, Oskar Schnappauf, Ivona Aksentijevich   +2 more
exaly   +5 more sources

Update on Pyrin Functions and Mechanisms of Familial Mediterranean Fever [PDF]

Frontiers in Microbiology, 2016
Mutations in the MEFV gene, which encodes the protein named pyrin (also called marenostrin or TRIM20), are associated with the autoinflammatory disease familial Mediterranean fever (FMF).
Gayane Manukyan, Rustam Aminov, Aminov Rustam   +2 more
exaly   +5 more sources

Pyrin- and CARD-only Proteins as Regulators of NLR Functions [PDF]

Frontiers in Immunology, 2013
Upon activation Nod-like receptors (NLRs) assemble into multi-protein complexes such as the NODosome and inflammasome. This process relies upon homo-domain interactions between the structurally related Pyrin and caspase-recruitment (CARD) domains and ...
Jonathan A Harton, Thi Hong Nga Le, Harton Jonathan A   +2 more
exaly   +5 more sources

The pyrin inflammasome, a leading actor in pediatric autoinflammatory diseases

Frontiers in Immunology
The activation of the pyrin inflammasome represents a highly intriguing mechanism employed by the innate immune system to effectively counteract pathogenic agents. Despite its key role in innate immunity, pyrin has also garnered significant attention due
Luciana Breda, Francesco Chiarelli
exaly   +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

Chest pain in children with familial Mediterranean fever

The Turkish Journal of Pediatrics, 2023
Background. Familial Mediterranean fever (FMF) is the most common and autosomal recessive inherited autoinflammatory disease. The most common signs and symptoms are fever, abdominal pain, chest pain, and arthritis.
Emine Nur Sunar-Yayla, Pelin Esmeray Şenol, Deniz Gezgin Yıldırım, Oğuz Söylemezoğlu   +3 more
doaj   +1 more source