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TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family
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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6164161/ https://www.ncbi.nlm.nih.gov/pubmed/30200294 http://dx.doi.org/10.3390/ijms19092585 |
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author | Rol, Nina Kurakula, Konda Babu Happé, Chris Bogaard, Harm Jan Goumans, Marie-José |
author_facet | Rol, Nina Kurakula, Konda Babu Happé, Chris Bogaard, Harm Jan Goumans, Marie-José |
author_sort | Rol, Nina |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-6164161 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-61641612018-10-10 TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family Rol, Nina Kurakula, Konda Babu Happé, Chris Bogaard, Harm Jan Goumans, Marie-José Int J Mol Sci 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. MDPI 2018-08-31 /pmc/articles/PMC6164161/ /pubmed/30200294 http://dx.doi.org/10.3390/ijms19092585 Text en © 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Rol, Nina Kurakula, Konda Babu Happé, Chris Bogaard, Harm Jan Goumans, Marie-José TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family |
title | TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family |
title_full | TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family |
title_fullStr | TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family |
title_full_unstemmed | TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family |
title_short | TGF-β and BMPR2 Signaling in PAH: Two Black Sheep in One Family |
title_sort | tgf-β and bmpr2 signaling in pah: two black sheep in one family |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6164161/ https://www.ncbi.nlm.nih.gov/pubmed/30200294 http://dx.doi.org/10.3390/ijms19092585 |
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