Article
The SPRY domain of Pyrin, mutated in familial Mediterranean fever patients, interacts with inflammasome components and inhibits proIL-1beta processing.
Cell death and differentiation - 1 Aug 2007
Papin S, Cuenin S, Agostini L, Martinon F, Werner S, Beer H-D, Grütter C, Grütter M, Tschopp J
Abstract excerpt
The autoinflammatory disorders Muckle-Wells syndrome, familial cold urtecaria and chronic infantile neurological cutaneous and articular syndrome are associated with mutations in the NALP3 (Cryopyrin) gene, which is the central platform of the proinflammatory caspase-1 activating complex, named the inflammasome. In patients with another autoinflammatory disorder, familial Mediterranean fever (FMF), mutations in...
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