Potassium channels in pulmonary arterial hypertension

• Published in: The European respiratory journal Vol. 46; no. 4; pp. 1167 - 1177
• Main Authors: Boucherat, Olivier, Chabot, Sophie, Antigny, Fabrice, Perros, Frédéric, Provencher, Steeve, Bonnet, Sébastien
• Format: Journal Article
• Language: English
• Published: England 01.10.2015
• Subjects: Animals; Bone Morphogenetic Proteins - metabolism; Cell Death; Cell Proliferation; Disease Progression; Genetic Predisposition to Disease; Humans; Hypertension, Pulmonary - metabolism; Kv1.5 Potassium Channel - metabolism; MicroRNAs - metabolism; Nerve Tissue Proteins - metabolism; Polymorphism, Single Nucleotide; Potassium Channels - metabolism; Potassium Channels, Tandem Pore Domain - metabolism; Protein Processing, Post-Translational; Pulmonary Artery - pathology; Signal Transduction; Thrombosis - physiopathology; Vasoconstriction
• ISSN: 0903-1936; 1399-3003
• DOI: 10.1183/13993003.00798-2015

Pulmonary arterial hypertension (PAH) is a devastating cardiopulmonary disorder with various origins. All forms of PAH share a common pulmonary arteriopathy characterised by vasoconstriction, remodelling of the pre-capillary pulmonary vessel wall, and in situ thrombosis. Although the pathogenesis of PAH is recognised as a complex and multifactorial process, there is growing evidence that potassium channels dysfunction in pulmonary artery smooth muscle cells is a hallmark of PAH. Besides regulating many physiological functions, reduced potassium channels expression and/or activity have significant effects on PAH establishment and progression. This review describes the molecular mechanisms and physiological consequences of potassium channel modulation. Special emphasis is placed on KCNA5 (Kv1.5) and KCNK3 (TASK1), which are considered to play a central role in determining pulmonary vascular tone and may represent attractive therapeutic targets in the treatment of PAH.