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Role of Bone Morphogenetic Protein Receptors in the Development of Pulmonary Arterial Hypertension

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Membrane Receptors, Channels and Transporters in Pulmonary Circulation

Part of the book series: Advances in Experimental Medicine and Biology ((volume 661))

Abstract

The identification of mutations in the bone morphogenetic protein (BMP) type II receptor in the majority of cases of familial pulmonary arterial hypertension (PAH) has provided a focus for researchers studying the complex pathobiology of this condition. Mutations are also found in a proportion of idiopathic PAH cases and it is now emerging that dysfunctional BMP signaling plays a role in other more common forms of PAH, even in the absence of mutations in the gene. Study of the role of BMP signaling in endothelial, smooth muscle cell, progenitor cell and inflammatory cell biology may reveal novel pathways lending themselves to therapeutic intervention in PAH. This chapter summarizes the present status of our understanding of the role of BMPR-II mutations in PAH and indicates future directions for research.

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Authors and Affiliations

  1. Department of Medicine, University of Cambridge School of Clinical Medicine, 157, Addenbrooke’s Hospital, Hills Road, Cambridge, CB2 2QQ, UK

    Nicholas W. Morrell

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  1. Nicholas W. Morrell
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Correspondence to Nicholas W. Morrell .

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Editors and Affiliations

  1. Departments of Medicine and Pharmacology, University of Illinois at Chicago, Chicago, 60612, USA

    Jason X. -J. Yuan

  2. Guy’s Hospital Campus, Dept. Physiology, King’s College London, London, SE1 1UL, United Kingdom

    Jeremy P. T. Ward

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© 2010 Humana Press, a part of Springer Science+Business Media, LLC

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Morrell, N.W. (2010). Role of Bone Morphogenetic Protein Receptors in the Development of Pulmonary Arterial Hypertension. In: Yuan, JJ., Ward, J. (eds) Membrane Receptors, Channels and Transporters in Pulmonary Circulation. Advances in Experimental Medicine and Biology, vol 661. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-500-2_16

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