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The promise of recombinant BMP ligands and other approaches targeting BMPR-II in the treatment of pulmonary arterial hypertension.

Published version
Peer-reviewed

Type

Article

Change log

Authors

Ormiston, Mark L 
Upton, Paul D 
Morrell, Nicholas W 

Abstract

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.

Description

Keywords

1102 Cardiorespiratory Medicine and Haematology, Biomedical, Basic Science, Lung, Biotechnology, Rare Diseases, Cardiovascular, 5.1 Pharmaceuticals, 2.1 Biological and endogenous factors

Journal Title

Glob Cardiol Sci Pract

Conference Name

Journal ISSN

2305-7823
2305-7823

Volume Title

2015

Publisher

Global Cardiology Science and Practice
Sponsorship
British Heart Foundation (None)
Medical Research Council (MR/K020919/1)
British Heart Foundation (None)
British Heart Foundation (None)
Medical Research Council (G1000847)
British Heart Foundation (None)
Medical Research Council (G0800784)
British Heart Foundation (PG/15/39/31519)