The role of endothelin-1 in pulmonary arterial hypertension.
The role of endothelin-1 in pulmonary arterial hypertension.
复制标题
DOI：
10.5339/gcsp.2014.29
复制
发表时间：
2014
期刊：
影响因子：
--
通讯作者：
Yacoub MH
中科院分区：
文献类型：
--
作者：
Chester AH;Yacoub MH
肺动脉高压(PAH)是一种罕见但使人衰弱的疾病，如果不治疗，它会迅速发展为右心衰竭，并最终死亡。为了了解这种疾病的发病机制，已经确定了PAH患者血管内皮释放的血管活性物质的轮廓、表达和作用的差异。其中，内皮素-1(ET-1)尤其令人感兴趣，因为它被认为是一种极其强大的血管收缩因子，也参与了血管重塑。将ET-1确定为药物干预的靶点，导致了许多化合物的发现，这些化合物可以阻断ET-1介导其作用的受体。该综述阐述了支持ET-1在疾病的发生和发展中的作用的证据，并回顾了ET-1受体拮抗剂治疗PAH的各种临床试验的数据。
Pulmonary arterial hypertension (PAH) is a rare but debilitating disease, which if left untreated rapidly progresses to right ventricular failure and eventually death. In the quest to understand the pathogenesis of this disease differences in the profile, expression and action of vasoactive substances released by the endothelium have been identified in patients with PAH. Of these, endothelin-1 (ET-1) is of particular interest since it is known to be an extremely powerful vasoconstrictor and also involved in vascular remodelling. Identification of ET-1 as a target for pharmacological intervention has lead to the discovery of a number of compounds that can block the receptors via which ET-1 mediates its effects. This review sets out the evidence in support of a role for ET-1 in the onset and progression of the disease and reviews the data from the various clinical trials of ET-1 receptor antagonists for the treatment of PAH.