Moreover, other regionalized vascular disorders such as hereditary hemorrhagic telangiectasia (HHT) and pulmonary arterial hypertension (PAH) are mainly caused by mutations in the BMP receptors ACVRL1 (encoding ALK1) or ENG (encoding Endoglin) and BMPR2 respectively [21–24]. Here, ENG is linked to pulmonary arterial hypertension.