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Figure 1. Involvement of proteases in pulmonary arterial remodeling in PH. (A) Schematic representation of a healthy arterial wall. (B) Arterial remodeling is accompanied by PASMC hyperplasia and ECM degradation and remodeling leading to the loss of vessel architecture. Immune cell-derived serine proteases can hydrolyze ECM proteins and modulate the bioavailability of cytokines, chemokines, and growth factors, thus regulating processes central to the development of vascular pathology. PAEC: Pulmonary arterial endothelial cells, PASMC: pulmonary arterial smooth muscle cells, advFB: adventitial fibroblasts, ECM: extracellular matrix, NE: neutrophil elastase, PR3: proteinase 3, CatG: cathepsin G. Graphical abstract has been created with BioRender.com.