Our findings identify a pyrin-inflammasome driven IL-18/IFNγ axis as a key signaling module of MKD-associated inflammation. This pathway may represent a novel target for therapeutic intervention in MKD.
Pyrin Inflammasome Activation Triggers an IL-18-Driven IFNγ Response in Mevalonate Kinase Deficiency
Niels S. van Heusden·Marianne Boes·Judith Jans·Daan K. J. Pieren·Nils Meijer·Charlotte Girard·Joost Frenkel·Bas Vastert·Isa Cuijpers·Isabelle Koné-Paut·Dörte Hamann·Angela Markovska·Cem Gabay·Erika Van Nieuwenhove