RNA-seq analysis of CRISPR/Cas9 generated human BMPR2 deficient endothelial cell lines harboring mutations characteristic for hereditary pulmonary hypertension (HPAH)

Balanced TGFß/BMP-signalling is essential for tissue formation and homeostasis. While gain in TGFß signaling is often found in diseases, the underlying cellular mechanisms remain poorly defined. Here we show, that the receptor BMPR2 serves as a central gatekeeper of this balance, highlighted by its deregulation in diseases such as pulmonary arterial hypertension (PAH). We show that BMPR2-deficient cells express genes indicative for altered biophysical properties including up-regulation of ECM proteins and integrins. Overall design: Endothelial cells (EAhy926) BMPR2wt and BMPR2?E2 and BMPR2KO were grown in a confluent monolayer for 3 days in a serum-rich growth media without media change. RNA was isolated using NucleoSpin® RNA from MACHEREY-NAGEL and submitted for RNA-Seq.