Patient-Specific iPSC-Derived Endothelial Cells Uncover Pathways that Protect against Pulmonary Hypertension in BMPR2 Mutation Carriers.
Sign Up to like & getrecommendations! Published in 2017 at "Cell stem cell"
DOI: 10.1016/j.stem.2016.08.019
Abstract: In familial pulmonary arterial hypertension (FPAH), the autosomal dominant disease-causing BMPR2 mutation is only 20% penetrant, suggesting that genetic variation provides modifiers that alleviate the disease. Here, we used comparison of induced pluripotent stem cell-derived endothelial… read more here.
Keywords: mutation carriers; bmpr2 mutation; ipsc ecs; endothelial cells ... See more keywords
Role of BMPR2 Mutation in Lung Organoid Differentiation
Sign Up to like & getrecommendations! Published in 2025 at "Biomedicines"
DOI: 10.3390/biomedicines13071623
Abstract: Background: The bone morphogenetic protein (BMP) signaling pathway is essential for lung development. BMP4, a key regulator, binds to type I (BMPR1) and type II (BMPR2) receptors to initiate downstream signaling. While the inactivation of… read more here.
Keywords: differentiation; bmpr2 mutation; lung; role bmpr2 ... See more keywords