Familial autoinflammation with neutrophilic dermatosis reveals a regulatory mechanism of pyrin activation

Pyrin responds to pathogen signals and loss of cellular homeostasis by forming an inflammasome complex that drives the cleavage and secretion of interleukin-1β (IL-1β). Mutations in the B30.2/SPRY domain cause pathogen-independent activation of pyrin and are responsible for the autoinflammatory dise...

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Published inScience translational medicine Vol. 8; no. 332; p. 332ra45
Main Authors Masters, Seth L, Lagou, Vasiliki, Jéru, Isabelle, Baker, Paul J, Van Eyck, Lien, Parry, David A, Lawless, Dylan, De Nardo, Dominic, Garcia-Perez, Josselyn E, Dagley, Laura F, Holley, Caroline L, Dooley, James, Moghaddas, Fiona, Pasciuto, Emanuela, Jeandel, Pierre-Yves, Sciot, Raf, Lyras, Dena, Webb, Andrew I, Nicholson, Sandra E, De Somer, Lien, van Nieuwenhove, Erika, Ruuth-Praz, Julia, Copin, Bruno, Cochet, Emmanuelle, Medlej-Hashim, Myrna, Megarbane, Andre, Schroder, Kate, Savic, Sinisa, Goris, An, Amselem, Serge, Wouters, Carine, Liston, Adrian
Format Journal Article
LanguageEnglish
Published United States 30.03.2016
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