Epigenetic Reactivation of Transcriptional Programs Orchestrating Fetal Lung Development in Human Pulmonary Hypertension

RNA sequencing in pulmonary artery fibroblasts (FBs) from adult human pulmonary arterial hypertension (PAH) and control lungs revealed 2460 differentially transcribed genes. Silencing of TBX4, TBX5, SOX9, or E1A-associated protein P300 by RNA interference or small-molecule compounds regressed PAH phenotypes and mesenchymal signatures in arterial FBs and smooth muscle cells.