ACVRL1 and pulmonary arterial hypertension: Due to the limited number of accessible PAH donors with ALK1 mutations, and to test whether LOF mutations in another component of the receptor complex would affect the response similarly, we tested BMPR2-mutated HMVECs derived from transplanted lungs of 3 PAH patients (Table 3), and found comparable regulation patterns to ALK1-mutated HMVECs for all 6 genes (Figs. 5a, S9a).