BMPR2 and pulmonary arterial hypertension: Indeed, genetic ablation of the BMPR2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension as indicated by the increased pulmonary and RV pressures, increased number of muscularized distal arteries, and thickening of medial layers, as well as the formation of plexiform lesions [40].