2. Academic Validation
  3. Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension

Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension

  • Biomolecules. 2020 Sep 1;10(9):1261. doi: 10.3390/biom10091261.
Hélène Le Ribeuz 1 2 3,  Véronique Capuano 1 2 3,  Barbara Girerd 1 2 3,  Marc Humbert 1 2 3,  David Montani 1 2 3,  Fabrice Antigny 1 2 3
Affiliations

Affiliations

  • 1 Faculté de Médecine, Université Paris-Saclay, 94270 Le Kremlin-Bicêtre, France.
  • 2 INSERM UMR_S 999, Hypertension Pulmonaire, Physiopathologie et Innovation Thérapeutique, Hôpital Marie Lannelongue, 92350 Le Plessis-Robinson, France.
  • 3 Assistance Publique-Hôpitaux de Paris (AP-HP), Service de Pneumologie et Soins Intensifs Respiratoires, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, 94270 Le Kremlin-Bicêtre, France.
PMID: 32882918 DOI: 10.3390/biom10091261
Abstract

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.

Keywords

ABCC8; ABCC9; K2P3.1; KCNA5; KCNK3; Kv1.5; SUR1; SUR2.

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