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

Abstract 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. The best characterized pathogenic mutations associated with FMF cluster in exon 10. Yet, mutations have been descri...

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Bibliographic Details
Main Authors: 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
Format: Article
Language:English
Published: Nature Publishing Group 2023-03-01
Series:Cell Death and Disease
Online Access:https://doi.org/10.1038/s41419-023-05745-9