We investigated the localization, expression, and function of SUR2A, SUR2B, and Kir6.1 in controls, PAH-human pulmonary arterial endothelial cells (hPAECs), and human pulmonary arterial smooth muscle cells (hPASMCs), and experimental models of pulmonary hypertension (PH). Here, KCNJ8 is linked to pulmonary arterial hypertension.