Gain-of-Function Pyrin Mutations Induce NLRP3 Protein -Independent Interleukin-1β Activation and Severe Autoinflammation in Mice

Missense mutations in the C-terminal B30.2 domain of pyrin cause familial Mediterranean fever (FMF), the most common Mendelian autoinflammatory disease. However, it remains controversial as to whether FMF is due to the loss of an inhibitor of inflammation or to the activity of a proinflammatory mole...

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