It has been demonstrated that EC derived from BMPR2 deficient patients presenting with PAH are more vulnerable to DNA damage, and that this is a BMPR2 mediated mechanism that is intriguingly specific to endothelial cells, though this may be explained by the differential regulation of cell survival signaling by BMPR2 in ECs and SMCs. This evidence concerns the gene BMPR2 and pulmonary arterial hypertension.