Article
The familial Mediterranean fever protein, pyrin, is cleaved by caspase-1 and activates NF-kappaB through its N-terminal fragment.
Blood - 1 Sept 2008
Chae Jae Jin, Wood Geryl, Richard Katharina, Jaffe Howard, Colburn Nona T, Masters Seth L, Gumucio Deborah L, Shoham Nitza G, Kastner Daniel L
Abstract excerpt
Familial Mediterranean fever (FMF) is an autoinflammatory disease caused by mutations in MEFV, which encodes a 781-amino acid protein denoted pyrin. We have previously shown that pyrin regulates caspase-1 activation and IL-1beta production through interaction of its N-terminal PYD motif with the ASC adapter protein, and also modulates IL-1beta production by interaction of its C-terminal B30.2 domain with the...
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