Fig. 7

Schematic diagram of the maintenance of RAD51 by the BMP9–ALK-1/BMPR2 signaling axis. In healthy individuals, RAD51 is maintained in the pulmonary endothelium as the BMP9–ALK-1/BMPR2 signaling axis downregulates miR-96 expression and prevents the destabilization of RAD51 mRNA. When the BMP9–ALK-1/BMPR2 signaling axis is deregulated, elevation of miR-96 leads to attenuation of RAD51. As a result, DNA damage accumulates in the pulmonary endothelium over several decades causing endothelial injury and dysfunction, and eventually triggers pulmonary vascular remodeling