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

International Journal of Molecular Sciences
Adam Andruska, Edda Spiekerkoetter

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.

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Citations

Feb 8, 2019·Pulmonary Circulation·Daniel GrinnanEdda Spiekerkoetter
Jun 7, 2019·Proceedings of the National Academy of Sciences of the United States of America·Jodie L Babitt
May 14, 2020·Expert Opinion on Pharmacotherapy·F Rana Olguntürk
Feb 6, 2020·American Journal of Physiology. Lung Cellular and Molecular Physiology·Carlyne D CoolNorbert F Voelkel
Jun 14, 2020·International Journal of Molecular Sciences·Maria Catalina Gomez-PuertoPeter Ten Dijke
Apr 28, 2020·American Journal of Respiratory Cell and Molecular Biology·Adam AndruskaEdda Spiekerkoetter
Nov 28, 2019·International Journal of Molecular Sciences·Ana I FernándezJavier Bermejo
Jan 6, 2021·Journal of Oral Biology and Craniofacial Research·Alieh FarshbafSeyed Abdolrahim Rezaee
Feb 14, 2021·International Journal of Molecular Sciences·Denise van UdenRudi W Hendriks
Jan 14, 2021·Biomedicines·Kondababu KurakulaMarie-José Goumans
Nov 26, 2020·Genes·Meghan M CirulisGreg Elliott
Dec 31, 2020·Genes·Svenja Dannewitz ProssedaEdda Spiekerkoetter
Oct 17, 2020·The European Respiratory Journal·Emilia M SwietlikChristopher J Rhodes
Oct 17, 2021·Chest·David F CondonVinicio A de Jesus Perez

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Methods Mentioned

BETA
glycation
biopsy
electron microscopy
ubiquitination
PAECs

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