Results 11 to 20 of about 88,038 (326)

CDC42 regulates PYRIN inflammasome assembly. [PDF]

Cell Reports, 2022
The PYRIN inflammasome pathway is part of the innate immune response against invading pathogens. Unprovoked continuous activation of the PYRIN inflammasome drives autoinflammation and underlies several autoinflammatory diseases, including familial Mediterranean fever (FMF) syndrome.
L. Spel, Léa Zaffalon, C. Hou, Nicaise Nganko, C. Chapuis, F. Martinon   +5 more
semanticscholar   +5 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

Function and mechanism of the pyrin inflammasome [PDF]

European Journal of Immunology, 2018
AbstractPyrin, encoded by the MEFV gene, is an intracellular pattern recognition receptor that assembles inflammasome complexes in response to pathogen infections. Mutations in the MEFV gene have been linked to autoinflammatory diseases such as familial Mediterranean fever (FMF) or pyrin‐associated autoinflammation with neutrophilic dermatosis (PAAND).
Petr Broz
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 ...
Hongnga T. Le, Hongnga T. Le, Jonathan A. Harton   +2 more
doaj   +2 more sources

HDAC6 mediates an aggresome-like mechanism for NLRP3 and pyrin inflammasome activation

Science, 2020
The MTOC is “speck”-tacular Inflammasome complexes are formed in response to pathogen-associated molecules. They initiate both the maturation of inflammatory cytokines and pyroptosis, a type of programmed cell death.
Venkat Giri Magupalli, Roberto Negro, Yuzi Tian   +2 more
exaly   +2 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
Saverio La Bella, Armando Di Ludovico, Giulia Di Donato, Ozge Basaran, Seza Ozen, Marco Gattorno, Francesco Chiarelli, Luciana Breda   +7 more
doaj   +2 more sources

Phosphoprotein phosphatase activity positively regulates oligomeric pyrin to trigger inflammasome assembly in phagocytes

mBio, 2023
Pyrin is a pattern-recognition receptor in phagocytes that triggers caspase-1 inflammasome assembly in response to bacterial toxins and effectors that inactivate RhoA.
Haleema S. Malik, Flora Magnotti, Nicole A. Loeven, Jose M. Delgado, Arminja N. Kettenbach, Thomas Henry, James B. Bliska   +6 more
doaj   +2 more sources

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

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

A dominant pathogenic MEFV mutation causes atypical pyrin-associated periodic syndromes

JCI Insight, 2023
Pyrin, a protein encoded by the MEFV gene, plays a vital role in innate immunity by sensing modifications in Rho GTPase and assembling the pyrin inflammasome, which in turn activates downstream immune responses.
Qintao Wang, Taijie Jin, Shan Jian, Xu Han, Hongmei Song, Qing Zhou, Xiaomin Yu   +6 more
doaj   +2 more sources

Pyrin Modulates the Intracellular Distribution of PSTPIP1. [PDF]

PLoS ONE, 2009
PSTPIP1 is a cytoskeleton-associated adaptor protein that links PEST-type phosphatases to their substrates. Mutations in PSTPIP1 cause PAPA syndrome (Pyogenic sterile Arthritis, Pyoderma gangrenosum, and Acne), an autoinflammatory disease.
Andrea L Waite, Philip Schaner, Neil Richards, Banu Balci-Peynircioglu, Seth L Masters, Susannah D Brydges, Michelle Fox, Arthur Hong, Engin Yilmaz, Daniel L Kastner, Ellis L Reinherz, Deborah L Gumucio   +11 more
doaj   +6 more sources