Expression of Caspase-1 variants induced ER stress
Pediatric Rheumatology(2015)
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.
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Key words
Endoplasmic Reticulum Stress, Unfold Protein Response, NLRP3 Inflammasome, Inflammasome Activation, Endoplasmic Reticulum Stress Response
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