The pyrin inflammasome, a leading actor in pediatric autoinflammatory diseases

S La Bella, A Di Ludovico, G Di Donato… - Frontiers in …, 2024 - frontiersin.org
The activation of the pyrin inflammasome represents a highly intriguing mechanism
employed by the innate immune system to effectively counteract pathogenic agents. Despite …

The pyrin inflammasome: from sensing RhoA GTPases-inhibiting toxins to triggering autoinflammatory syndromes

Y Jamilloux, F Magnotti, A Belot… - Pathogens and …, 2018 - academic.oup.com
Numerous pathogens including Clostridium difficile and Yersinia pestis have evolved toxins
or effectors targeting GTPases from the RhoA subfamily (RhoA/B/C) to inhibit or hijack the …

Recent Insights in Pyrin Inflammasome Activation: Identifying Potential Novel Therapeutic Approaches in Pyrin-Associated Autoinflammatory Syndromes

F Wouters, J Bogie, A Wullaert… - Journal of Clinical …, 2024 - Springer
Pyrin is a cytosolic protein encoded by the MEFV gene, predominantly expressed in innate
immune cells. Upon activation, it forms an inflammasome, a multimolecular complex that …

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

D Chirita, P Bronnec, F Magnotti, S Dalmon… - Cell Death & …, 2023 - nature.com
Abstract Familial Mediterranean Fever (FMF) is the most common monogenic
autoinflammatory disorder. FMF is caused by mutations in the MEFV gene, encoding pyrin …

The pyrin inflammasome in health and disease

O Schnappauf, JJ Chae, DL Kastner… - Frontiers in …, 2019 - frontiersin.org
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 …

Function and mechanism of the pyrin inflammasome

R Heilig, P Broz - European journal of immunology, 2018 - Wiley Online Library
Pyrin, encoded by the MEFV gene, is an intracellular pattern recognition receptor that
assembles inflammasome complexes in response to pathogen infections. Mutations in the …

Ribotoxic stress through p38 mitogen-activated protein kinase activates in vitro the human pyrin inflammasome

JW Yu, A Farias, I Hwang, T Fernandes-Alnemri… - Journal of Biological …, 2013 - ASBMB
Human pyrin with gain-of-function mutations in its B30. 2/SPRY domain causes the
autoinflammatory disease familial Mediterranean fever by assembling an ASC-dependent …

[HTML][HTML] CDC42 regulates PYRIN inflammasome assembly

L Spel, L Zaffalon, C Hou, N Nganko, C Chapuis… - Cell Reports, 2022 - cell.com
The PYRIN inflammasome pathway is part of the innate immune response against invading
pathogens. Unprovoked continuous activation of the PYRIN inflammasome drives …

Steroid hormone catabolites activate the pyrin inflammasome through a non-canonical mechanism

F Magnotti, D Chirita, S Dalmon, A Martin, P Bronnec… - Cell reports, 2022 - cell.com
The pyrin inflammasome acts as a guard of RhoA GTPases and is central to immune
defenses against RhoA-manipulating pathogens. Pyrin activation proceeds in two steps. Yet …

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

HS Malik, F Magnotti, NA Loeven, JM Delgado… - Mbio, 2023 - Am Soc Microbiol
Pyrin is a pattern-recognition receptor in phagocytes that triggers caspase-1 inflammasome
assembly in response to bacterial toxins and effectors that inactivate RhoA. Pyrin contains …