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Dysfunctional inflammasome in Schnitzler's syndrome
Rheumatology, 08/26/09
Pizzirani C et al. – In patients with Schnitzler's syndrome (SS), there is a derangement of the inflammasome and IL-1beta processing.
Methods- PBMCs were examined for the ability to secrete IL-1beta and IL-18, expression and function of P2X7 receptor and expression of apoptosis-associated speck-like protein containing a caspase recruitment domaine (ASC) and NOD-like receptor protein 3 (NLRP3) before and after tx with steroids
- Extracellular ATP blood levels were determined by luciferase assay
- Expression of inflammasome components was measured by RT-PCR and Western blotting
- PBMCs had a high, spontaneous and LPS-stimulated IL-1beta release, but a low response to stimulation with the P2X7 agonist, benzoyl ATP
- P2X7 expression was several-fold increased, whereas ASC expression was dramatically decreased compared with PBMCs from healthy controls
- NLRP3 expression was unchanged
- Prednisone tx induced remission of clinical symptoms and normalized IL-1beta secretion and P2X7 and ASC expression
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