Antigny and colleagues [39] demonstrated decreased lung and pulmonary artery expression of KCNK3 in freshly isolated PASMCs from IPAH patients and HPAH patients with BMPR2 variants compared to unaffected controls; and decreased channel conductance in IPAH vs. control PASMCs. The gene discussed is BMPR2; the disease is heritable pulmonary arterial hypertension.