RT Journal Article SR Electronic(1) A1 Ormiston, Mark L. A1 Upton, Paul D. A1 Li, Wei A1 Morrell, Nicholas W.YR 2015 T1 The promise of recombinant BMP ligands and other approaches targeting BMPR-II in the treatment of pulmonary arterial hypertension JF Global Cardiology Science and Practice, VO 2015 IS 4 OP SP 47 DO https://doi.org/10.5339/gcsp.2015.47 PB Hamad bin Khalifa University Press (HBKU Press), SN 2305-7823, AB Human genetic discoveries offer a powerful method to implicate pathways of major importance to disease pathobiology and hence provide targets for pharmacological intervention. The genetics of pulmonary arterial hypertension (PAH) strongly implicates loss-of-function of the bone morphogenetic protein type II receptor (BMPR-II) signalling pathway and moreover implicates the endothelial cell as a central cell type involved in disease initiation. We and others have described several approaches to restore BMPR-II function in genetic and non-genetic forms of PAH. Of these, supplementation of endothelial BMP9/10 signalling with exogenous recombinant ligand has been shown to hold considerable promise as a novel large molecule biopharmaceutical therapy. Here, we describe the mechanism of action and discuss potential additional effects of BMP ligand therapy., UL https://www.qscience.com/content/journals/10.5339/gcsp.2015.47