Fig. 5

Schematic Representation of the mechanism of CP40-KK in the therapy of PAH Pulmonary arterial hypertension (PAH) is triggered by macrophage–NLRP3 activation and can be blocked by CP40-KK. Proposed mechanism of NLRP3 activation in PAH: the activation of the NLRP3 inflammasome pathway in rat lung is triggered by complement C3a in plasma. NLRP3 inflammasome assembles with apoptosis-associated speck-like protein (ASC), leading to release of cleaved-IL1β and IL18. Cleaved-IL1β and IL18 could promote PASMCs proliferation, contributing to pulmonary vascular remodeling. This study proposed one therapeutic approach to cease the inflammatory process: CP40-KK, a selective complement C3 inhibitor, preventing complement C3 activation. ASC: apoptosis-associated speck-like protein