Abstract
Caspase-1 is a proinflammatory enzyme that is activated by the NLRP3 inflammasome in response to endoplasmic reticulum (ER) stress independent of the classical unfolded protein response. This finding linked ER stress to chronic inflammatory diseases. In patients suffering from unexplained recurrent febrile episodes we detected several genetic variants of CASP1 leading to reduced enzymatic activity due to destabilization of the caspase-1 dimer interface.
Highlights
Caspase-1 is a proinflammatory enzyme that is activated by the NLRP3 inflammasome in response to endoplasmic reticulum (ER) stress independent of the classical unfolded protein response
In patients suffering from unexplained recurrent febrile episodes we detected several genetic variants of CASP1 leading to reduced enzymatic activity due to destabilization of the caspase-1 dimer interface
University Hospital Carl Gustav Carus, Department of Pediatrics, Dresden, Germany
Summary
Caspase-1 is a proinflammatory enzyme that is activated by the NLRP3 inflammasome in response to endoplasmic reticulum (ER) stress independent of the classical unfolded protein response. This finding linked ER stress to chronic inflammatory diseases. In patients suffering from unexplained recurrent febrile episodes we detected several genetic variants of CASP1 leading to reduced enzymatic activity due to destabilization of the caspase-1 dimer interface
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