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

Archive ouverte | Masters, Seth | CCSD

International audience. 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...

Enhanced cGAS-STING-dependent interferon signaling associated with mutations in ATAD3A

Archive ouverte | Lepelley, Alice | CCSD

International audience. Mitochondrial DNA (mtDNA) has been suggested to drive immune system activation, but the induction of interferon signaling by mtDNA has not been demonstrated in a Mendelian mitochondrial disea...

Human OTULIN haploinsufficiency impairs cell-intrinsic immunity to staphylococcal α-toxin

Archive ouverte | Spaan, András | CCSD

International audience. The molecular basis of interindividual clinical variability upon infection with Staphylococcus aureus is unclear. We describe patients with haploinsufficiency for the linear deubiquitinase OT...

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