Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension
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...
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| Format: | Article |
| Language: | English |
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MDPI AG
2020-09-01
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| Series: | Biomolecules |
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| Online Access: | https://www.mdpi.com/2218-273X/10/9/1261 |
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| author | Hélène Le Ribeuz Véronique Capuano Barbara Girerd Marc Humbert David Montani Fabrice Antigny |
| author_facet | Hélène Le Ribeuz Véronique Capuano Barbara Girerd Marc Humbert David Montani Fabrice Antigny |
| author_sort | Hélène Le Ribeuz |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T16:39:00Z |
| format | Article |
| id | doaj.art-a7f521eeb62c48e99ab90d8eb0197ea4 |
| institution | Directory Open Access Journal |
| issn | 2218-273X |
| language | English |
| last_indexed | 2024-03-10T16:39:00Z |
| publishDate | 2020-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomolecules |
| spelling | doaj.art-a7f521eeb62c48e99ab90d8eb0197ea42023-11-20T12:10:20ZengMDPI AGBiomolecules2218-273X2020-09-01109126110.3390/biom10091261Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial HypertensionHélène Le Ribeuz0Véronique Capuano1Barbara Girerd2Marc Humbert3David Montani4Fabrice Antigny5Faculté de Médecine, Université Paris-Saclay, 94270 Le Kremlin-Bicêtre, FranceFaculté de Médecine, Université Paris-Saclay, 94270 Le Kremlin-Bicêtre, FranceFaculté de Médecine, Université Paris-Saclay, 94270 Le Kremlin-Bicêtre, FranceFaculté de Médecine, Université Paris-Saclay, 94270 Le Kremlin-Bicêtre, FranceFaculté de Médecine, Université Paris-Saclay, 94270 Le Kremlin-Bicêtre, FranceFaculté de Médecine, Université Paris-Saclay, 94270 Le Kremlin-Bicêtre, FrancePulmonary 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.https://www.mdpi.com/2218-273X/10/9/1261KCNK3ABCC8KCNA5ABCC9K2P3.1SUR1 |
| spellingShingle | Hélène Le Ribeuz Véronique Capuano Barbara Girerd Marc Humbert David Montani Fabrice Antigny Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension Biomolecules KCNK3 ABCC8 KCNA5 ABCC9 K2P3.1 SUR1 |
| title | Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension |
| title_full | Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension |
| title_fullStr | Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension |
| title_full_unstemmed | Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension |
| title_short | Implication of Potassium Channels in the Pathophysiology of Pulmonary Arterial Hypertension |
| title_sort | implication of potassium channels in the pathophysiology of pulmonary arterial hypertension |
| topic | KCNK3 ABCC8 KCNA5 ABCC9 K2P3.1 SUR1 |
| url | https://www.mdpi.com/2218-273X/10/9/1261 |
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