Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension

Andruska, Adam; Spiekerkoetter, Edda

doi:10.3390/ijms19092499

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Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension

Adam Andruska

¹ and

Edda Spiekerkoetter

^1,2,3,*

Department of Medicine, Division of Pulmonary and Critical Care, Stanford University, Stanford, CA 94305, USA

Wall Center for Pulmonary Vascular Disease, Stanford University, Stanford, CA 94305, USA

Cardiovascular Institute, Stanford University, Stanford, CA 94305, USA

Author to whom correspondence should be addressed.

Int. J. Mol. Sci. 2018, 19(9), 2499; https://doi.org/10.3390/ijms19092499

Received: 1 August 2018 / Revised: 17 August 2018 / Accepted: 18 August 2018 / Published: 24 August 2018

(This article belongs to the Special Issue Molecular Research on Pulmonary Hypertension)

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Abstract

Since its association with familial pulmonary arterial hypertension (PAH) in 2000, Bone Morphogenetic Protein Receptor II (BMPR2) and its related signaling pathway have become recognized as a key regulator of pulmonary vascular homeostasis. Herein, we define BMPR2 deficiency as either an inactivation of the receptor, decreased receptor expression, or an impairment of the receptor’s downstream signaling pathway. Although traditionally the phenotypic consequences of BMPR2 deficiency in PAH have been thought to be limited to the pulmonary vasculature, there is evidence that abnormalities in BMPR2 signaling may have consequences in many other organ systems and cellular compartments. Revisiting how BMPR2 functions throughout health and disease in cells and organs beyond the lung vasculature may provide insight into the contribution of these organ systems to PAH pathogenesis as well as the potential systemic manifestation of PAH. Here we review our knowledge of the consequences of BMPR2 deficiency across multiple organ systems. View Full-Text

Keywords: pulmonary hypertension; Bone Morphogenetic Protein Receptor II; vascular disease; genetic predisposition to disease

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MDPI and ACS Style

Andruska, A.; Spiekerkoetter, E. Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension. Int. J. Mol. Sci. 2018, 19, 2499. https://doi.org/10.3390/ijms19092499

AMA Style

Andruska A, Spiekerkoetter E. Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension. International Journal of Molecular Sciences. 2018; 19(9):2499. https://doi.org/10.3390/ijms19092499

Chicago/Turabian Style

Andruska, Adam, and Edda Spiekerkoetter. 2018. "Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension" International Journal of Molecular Sciences 19, no. 9: 2499. https://doi.org/10.3390/ijms19092499

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Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension

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