Ontology highlight
ABSTRACT:
SUBMITTER: Magnotti F
PROVIDER: S-EPMC6835204 | biostudies-literature | 2019 Nov
REPOSITORIES: biostudies-literature
Magnotti Flora F Lefeuvre Lucie L Benezech Sarah S Malsot Tiphaine T Waeckel Louis L Martin Amandine A Kerever Sébastien S Chirita Daria D Desjonqueres Marine M Duquesne Agnès A Gerfaud-Valentin Mathieu M Laurent Audrey A Sève Pascal P Popoff Michel-Robert MR Walzer Thierry T Belot Alexandre A Jamilloux Yvan Y Henry Thomas T
EMBO molecular medicine 20191007 11
Familial Mediterranean fever (FMF) is the most frequent hereditary systemic autoinflammatory syndrome. FMF is usually caused by biallelic mutations in the MEFV gene, encoding Pyrin. Conclusive genetic evidence lacks for about 30% of patients diagnosed with clinical FMF. Pyrin is an inflammasome sensor maintained inactive by two kinases (PKN1/2). The consequences of MEFV mutations on inflammasome activation are still poorly understood. Here, we demonstrate that PKC superfamily inhibitors trigger ...[more]