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

ABSTRACT: Balanced TGFβ/BMP-signalling is essential for tissue formation and homeostasis. While gain in TGFβ 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.

ORGANISM(S): Homo sapiens

PROVIDER: GSE135312 | GEO | 2019/11/14

REPOSITORIES: GEO

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