| Summary: | Pulmonary arterial hypertension (PAH) is a rare and severe cardiopulmonary disease without curative treatments. PAH is a multifactorial disease that involves genetic predisposition, epigenetic factors, and environmental factors (drugs, toxins, viruses, hypoxia, and inflammation), which contribute to the initiation or development of irreversible remodeling of the pulmonary vessels. The recent identification of loss-of-function mutations in <i>KCNK3</i> (KCNK3 or TASK-1) and <i>ABCC8</i> (SUR1), or gain-of-function mutations in <i>ABCC9</i> (SUR2), as well as polymorphisms in <i>KCNA5</i> (Kv1.5), which encode two potassium (K<sup>+</sup>) channels and two K<sup>+</sup> channel regulatory subunits, has revived the interest of ion channels in PAH. This review focuses on KCNK3, SUR1, SUR2, and Kv1.5 channels in pulmonary vasculature and discusses their pathophysiological contribution to and therapeutic potential in PAH.
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