Article
Pyrin activates the ASC pyroptosome in response to engagement by autoinflammatory PSTPIP1 mutants.
Molecular cell - 26 Oct 2007
Yu Je-Wook, Fernandes-Alnemri Teresa, Datta Pinaki, Wu Jianghong, Juliana Christine, Solorzano Leobaldo, McCormick Margaret, Zhang ZhiJia, Alnemri Emad S
Abstract excerpt
The molecular mechanism by which mutations in the cytoskeleton-organizing protein PSTPIP1 cause the autoinflammatory PAPA syndrome is still elusive. Here, we demonstrate that PSTPIP1 requires the familial Mediterranean fever protein pyrin to assemble the ASC pyroptosome, a molecular platform that recruits and activates caspase-1. We provide evidence that pyrin is a cytosolic receptor for PSTPIP1. Pyrin exists as...
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