PDF(Pubmed)
Abstract：
Pulmonary hypertension (PH) is a complex cardiopulmonary disorder impacting the lung vasculature, resulting in increased pulmonary vascular resistance that leads to right ventricular dysfunction. Pulmonary hypertension comprises of 5 groups (PH group 1 to 5) where group 1 pulmonary arterial hypertension (PAH), results from alterations that directly affect the pulmonary arteries. Although PAH has a complex pathophysiology that is not completely understood, it is known to be a multifactorial disease that results from a combination of genetic, epigenetic and environmental factors, leading to a varied range of symptoms in PAH patients. PAH does not have a cure, its incidence and prevalence continue to increase every year, resulting in higher morbidity and mortality rates. In this review, we discuss the different pathologic mechanisms with a focus on epigenetic modifications and their roles in the development and progression of PAH. These modifications include DNA methylation, histone modifications, and microRNA dysregulation. Understanding these epigenetic modifications will improve our understanding of PAH and unveil novel therapeutic targets, thus steering research toward innovative treatment strategies.
摘要：
肺动脉高压（PH）是一种影响肺血管系统的复杂心肺疾病，导致肺血管阻力增加，导致右心室功能障碍。肺动脉高压包括5组（PH组1至5），其中组1肺动脉高压（PAH），直接影响肺动脉的改变。尽管PAH具有复杂的病理生理学尚未完全了解，它被认为是一种多因素的疾病，由遗传的组合，表观遗传和环境因素，导致PAH患者出现各种不同的症状。PAH没有治愈方法，其发病率和患病率逐年上升，导致更高的发病率和死亡率。在这次审查中,我们讨论了不同的病理机制，重点是表观遗传修饰及其在PAH发生和发展中的作用。这些修饰包括DNA甲基化，组蛋白修饰，和microRNA失调。了解这些表观遗传修饰将提高我们对PAH的理解，并揭示新的治疗靶点。从而将研究转向创新的治疗策略。
