Published in:
Open Access
01-11-2013 | Meeting abstract
PW03-027 - CASP1 variants and live cell imaging
Authors:
S Rabe, MC Heymann, S Russ, S Winkler, J Roesler, A Rösen-Wolff, SR Hofmann
Published in:
Pediatric Rheumatology
|
Special Issue 1/2013
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Excerpt
Patients with unexplained recurrent febrile episodes and CASP1 variants suffer from systemic sterile inflammation despite reduced secretion of IL-1ß. As previously demonstrated by our group CASP1 variants lead to reduced enzymatic activity of procaspase-1 by destabilizing the tertiary structure of the caspase-1 tetramer. A possible explanation for an alternative pro-inflammatory pathway has been provided by Lamkanfi and colleagues indicating an association between enzymatically inactive procaspase-1 and receptor interacting protein kinase 2 (RIP2) leading to NF-kB activation. …