Department of Medicine, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Department of Medicine, Perelman School at the University of Pennsylvania, Philadelphia, PA, USA.
Commun Biol. 2023 Aug 9;6(1):826. doi: 10.1038/s42003-023-05193-3.
Portopulmonary hypertension (PoPH) is a type of pulmonary vascular disease due to portal hypertension that exhibits high morbidity and mortality. The mechanisms driving disease are unknown, and transcriptional characteristics unique to the PoPH liver remain unexplored. Here, we apply single nuclear RNA sequencing to compare cirrhotic livers from patients with and without PoPH. We identify characteristics unique to PoPH in cells surrounding the central hepatic vein, including increased growth differentiation factor signaling, enrichment of the arginine biosynthesis pathway, and differential expression of the bone morphogenic protein type II receptor and estrogen receptor type I genes. These results provide insight into the transcriptomic characteristics of the PoPH liver and mechanisms by which PoPH cellular dysfunction might contribute to pulmonary vascular remodeling.
肝肺高压（PoPH）是一种由门静脉高压引起的肺血管疾病，其发病率和死亡率都很高。导致这种疾病的机制尚不清楚，而 PoPH 肝脏特有的转录特征仍未被探索。在这里，我们应用单细胞核 RNA 测序来比较患有和不患有 PoPH 的患者的肝硬化肝脏。我们在围绕中央肝静脉的细胞中发现了 PoPH 特有的特征，包括生长分化因子信号的增加、精氨酸生物合成途径的富集，以及骨形态发生蛋白 II 型受体和雌激素受体 I 基因的差异表达。这些结果为 PoPH 肝脏的转录组特征以及 PoPH 细胞功能障碍可能导致肺血管重塑的机制提供了深入了解。
