Abstract
Mutations in the genes encoding pyrin and mevalonate kinase (MVK) cause the autoinflammatory diseases familial Mediterranean fever (FMF) and hyperimmunoglobulinemia D syndrome (HIDS), respectively. The inflammation of both diseases is mediated by interleukin-1β (IL-1β). Recently it has been reported that pyrin forms an inflammasome, a multiprotein complex that mediates the maturation of IL-1β by activating caspase-1, in response to bacterial modifications of RhoA. However, the precise molecular mechanism by which the pyrin inflammasome is activated is unknown.
Highlights
Mutations in the genes encoding pyrin and mevalonate kinase (MVK) cause the autoinflammatory diseases familial Mediterranean fever (FMF) and hyperimmunoglobulinemia D syndrome (HIDS), respectively. The inflammation of both diseases is mediated by interleukin-1b (IL-1b)
It has been reported that pyrin forms an inflammasome, a multiprotein complex that mediates the maturation of IL-1b by activating caspase[1], in response to bacterial modifications of RhoA
ConclusionThese data directly implicate Rho GTPase in the regulation of the pyrin inflammasome, and suggest that this pathway is important in hyperimmunoglobulinemia D syndrome (HIDS)
Summary
Mutations in the genes encoding pyrin and mevalonate kinase (MVK) cause the autoinflammatory diseases familial Mediterranean fever (FMF) and hyperimmunoglobulinemia D syndrome (HIDS), respectively. The inflammation of both diseases is mediated by interleukin-1b (IL-1b). It has been reported that pyrin forms an inflammasome, a multiprotein complex that mediates the maturation of IL-1b by activating caspase[1], in response to bacterial modifications of RhoA. The precise molecular mechanism by which the pyrin inflammasome is activated is unknown
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