Article
Familial Mediterranean fever mutations lift the obligatory requirement for microtubules in Pyrin inflammasome activation.
Proceedings of the National Academy of Sciences of the United States of America - 13 Dec 2016
Van Gorp Hanne, Saavedra Pedro H V, de Vasconcelos Nathalia M, Van Opdenbosch Nina, Vande Walle Lieselotte, Matusiak Magdalena, Prencipe Giusi, Insalaco Antonella, Van Hauwermeiren Filip, Demon Dieter, Bogaert Delfien J, Dullaers Melissa, De Baere Elfride, Hochepied Tino, Dehoorne Joke, Vermaelen Karim Y, Haerynck Filomeen, De Benedetti Fabrizio, Lamkanfi Mohamed
Abstract excerpt
Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease worldwide. It is caused by mutations in the inflammasome adaptor Pyrin, but how FMF mutations alter signaling in FMF patients is unknown. Herein, we establish Clostridium difficile and its enterotoxin A (TcdA) as Pyrin-activating agents and show that wild-type and FMF Pyrin are differentially controlled by microtubules....
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