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Bone morphogenetic proteins, genetics and the pathophysiology of primary pulmonary hypertension

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Abstract

Several recent papers have shown that both familial primary pulmonary hypertension (FPPH) and sporadic primary pulmonary hypertension (PPH) may have a common etiology that is associated with the inheritance and/or spontaneous development of germline mutations in the bone morphogenetic protein receptor (BMPR) type II gene. Because BMPR-II is a ubiquitously expressed receptor for a family of secreted growth factors known as the bone morphogenetic proteins (BMPs), these findings suggest that BMPs play an important role in the maintenance of normal pulmonary vascular physiology. In the present commentary we discuss the implications of these findings in the context of BMP receptor biology, and relate these data to the genetics and pulmonary pathophysiology of patients with PPH.

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Figure 1

Abbreviations

ActR:

activin receptor

Alk:

activin receptor-like kinase

BMP:

bone morphogenetic protein

BMPR:

bone morphogenetic protein receptor

FPPH:

familial primary pulmonary hypertension

PPH:

primary pulmonary hypertension

TGF:

transforming growth factor.

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Authors and Affiliations

  1. Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA

    Mark De Caestecker & Barbara Meyrick

  2. Department of Pathology, Vanderbilt University Medical Center, Nashville, Tennessee, USA

    Barbara Meyrick

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  1. Mark De Caestecker
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  2. Barbara Meyrick
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Correspondence to Barbara Meyrick.

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Caestecker, M.D., Meyrick, B. Bone morphogenetic proteins, genetics and the pathophysiology of primary pulmonary hypertension. Respir Res 2, 193 (2001). https://doi.org/10.1186/rr57

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