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Article

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

PLoS biology - 1 Nov 2022

Mangan Matthew S J, Gorki Friederike, Krause Karoline, Heinz Alexander, Pankow Anne, Ebert Thomas, Jahn Dieter, Hiller Karsten, Hornung Veit, Maurer Marcus, Schmidt Florian I, Gerhard Ralf, Latz Eicke

Abstract excerpt

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. Gain-of-function mutations in the MEFV gene encoding Pyrin cause autoinflammatory disorders, such as familial Mediterranean fever (FMF) and Pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND). To...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 36342970
DOI: 10.1371/journal.pbio.3001351
PMCID: PMC9671422

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