NLRP3 inflammasome

Inflammasome sensor — activated by mitochondrial ROS

Expression: Hyperactivated
Evidence level: strong

Role in pathogenesis

The NLRP3 inflammasome is activated downstream of mitochondrial ROS generation in TRAPS. NLRP3 assembles with ASC and activates caspase-1, which cleaves pro-IL-1β into active IL-1β. Unlike CAPS, there are no NLRP3 mutations — the inflammasome is activated by upstream signals from the misfolded TNFR1.

Sources (2)

DetailsBulua AC et al. (2011) Mitochondrial reactive oxygen species promote production of proinflammatory cytokines and are elevated in TNFR1-associated periodic syndrome (TRAPS) · J Exp MedDOI ↗

DetailsCudrici C et al. (2020) Revisiting TNF Receptor-Associated Periodic Syndrome (TRAPS): Current Perspectives · Int J Mol SciDOI ↗