Epigenome-wide profiling uncovers novel pathway networks in human pulmonary arterial hypertension [pul31]

Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease that culminates in right heart failure. Vascular pathology in PH is characterized by pulmonary vasoconstriction and progressive vascular remodeling processes that affects all layers of the vascular wall (intima, media and adventitia). Our objective was to profile and analyze the differential gene expression signatures between the cells isolated from normal and idiopathic PAH patients. We generated vascular cell-specific transcriptome profiles from the adventitial fibroblasts (PAAF) isolated ex vivo from the dissected human pulmonary arteries of normal donor and PAH lungs using paired-end RNA-sequencing.肺动脉高压（Pulmonary arterial hypertension, PAH）是一种进展性肺血管疾病，最终可进展为右心衰竭。肺动脉高压的血管病理特征表现为肺血管收缩与进行性血管重构，该过程累及血管壁全层（内膜、中膜与外膜）。本研究旨在描绘并分析正常供体与特发性肺动脉高压（idiopathic PAH）患者来源细胞间的差异基因表达特征。我们采用双端RNA测序（paired-end RNA-sequencing）技术，对从正常供体及PAH患者肺部解剖分离的人肺动脉中离体分离得到的外膜成纤维细胞（adventitial fibroblasts, PAAF）构建了血管细胞特异性转录组图谱。