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Int. J. Mol. Sci. 2018, 19(9), 2585; https://doi.org/10.3390/ijms19092585

TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family

1
Department of Pulmonology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam Cardiovascular Sciences, 1081HV Amsterdam, The Netherlands
2
Department of Physiology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam Cardiovascular Sciences, 1081HV Amsterdam, The Netherlands
3
Department of Cell and Chemical Biology, Leiden University Medical Center, 2333ZA Leiden, The Netherlands
These authors contributed equally to this work.
*
Author to whom correspondence should be addressed.
Received: 27 July 2018 / Revised: 21 August 2018 / Accepted: 23 August 2018 / Published: 31 August 2018
(This article belongs to the Special Issue TGF-Beta Super Family Signaling)
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Abstract

Knowledge pertaining to the involvement of transforming growth factor β (TGF-β) and bone morphogenetic protein (BMP) signaling in pulmonary arterial hypertension (PAH) is continuously increasing. There is a growing understanding of the function of individual components involved in the pathway, but a clear synthesis of how these interact in PAH is currently lacking. Most of the focus has been on signaling downstream of BMPR2, but it is imperative to include the role of TGF-β signaling in PAH. This review gives a state of the art overview of disturbed signaling through the receptors of the TGF-β family with respect to vascular remodeling and cardiac effects as observed in PAH. Recent (pre)-clinical studies in which these two pathways were targeted will be discussed with an extended view on cardiovascular research fields outside of PAH, indicating novel future perspectives. View Full-Text
Keywords: pulmonary arterial hypertension; transforming growth factor β; bone morphogenetic protein; signaling; pathophysiology; treatment pulmonary arterial hypertension; transforming growth factor β; bone morphogenetic protein; signaling; pathophysiology; treatment
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).
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Rol, N.; Kurakula, K.B.; Happé, C.; Bogaard, H.J.; Goumans, M.-J. TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family. Int. J. Mol. Sci. 2018, 19, 2585.

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