Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension

Biomolecules

Hélène Le Ribeuz¹  David Montani¹  Barbara Girerd¹  Marc Humbert¹  Véronique Capuano¹  Fabrice Antigny¹ 

[1] Faculté de Médecine, Université Paris-Saclay, 94270 Le Kremlin-Bicêtre, France;

关键词: KCNK3;    ABCC8;    KCNA5;    ABCC9;    K2P3.1;    SUR1;   

DOI  :  10.3390/biom10091261

来源: DOAJ

【 摘 要 】

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 KCNK3 (KCNK3 or TASK-1) and ABCC8 (SUR1), or gain-of-function mutations in ABCC9 (SUR2), as well as polymorphisms in KCNA5 (Kv1.5), which encode two potassium (K⁺) channels and two K⁺ 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.

【 授权许可】

Unknown