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

Nina Rol, Konda Babu Kurakula, Chris Happé, Harm Jan Bogaard, Marie José Goumans*

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review


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.

Original languageEnglish
Article number2585
JournalInternational Journal of Molecular Sciences
Issue number9
Publication statusPublished - 31 Aug 2018

Cite this