MASP-2 and MASP-3 inhibitors block complement activation, inflammation, and microvascular stasis in a murine model of vaso-occlusion in sickle cell disease

Patients with sickle cell disease (SCD) have ongoing hemolysis that promotes endothelial injury, complement activation, inflammation, vaso-occlusion, ischemia-reperfusion pathophysiology, and pain. Complement activation markers are increased in SCD in steady-state and further increased during vaso-occlusive crisis (VOC). However, the mechanisms driving complement activation in SCD have not been completely elucidated. Ischemia-reperfusion and heme released from hemoglobin during hemolysis, events that characterize SCD pathophysiology, can activate the lectin pathway (LP) and alternative pathway (AP), respectively.

Source: Translational Research - July 22, 2022 Category: Research Authors: John D. Belcher, Julia Nguyen, Chunsheng Chen, Fuad Abdulla, Ruan Conglin, Zalaya K. Ivy, Jason Cummings, Thomas Dudler, Gregory M. Vercellotti Source Type: research