Sex differences in the proliferation of pulmonary artery endothelial cells: implications for plexiform arteriopathy.

The sex-biased disease pulmonary arterial hypertension (PAH) is characterized by the proliferation and overgrowth of dysfunctional pulmonary artery endothelial cells (PAECs). During inflammation associated with PAH, granzyme B cleaves intersectin-1 to produce N-terminal (EH) and C-terminal (SH3A-E) protein fragments. In a murine model of PAH, EH triggers plexiform arteriopathy via p38-ELK1-c-Fos signaling.