ACVRL1 and idiopathic pulmonary arterial hypertension: Besides that, other hereditary causes of PAH involve TGF-β superfamily genes including ALK1/ACVRL1 (a heterodimeric partner of BMPR2), BMP9 (a BMPR2 family member), ENG (a coreceptor for BMPR2 signaling), and SMAD1, 4, and 9 (downstream BMP signaling molecules) have been linked to both HPAH and IPAH [7].