期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 19, 期 9, 页码 -出版社
MDPI
DOI: 10.3390/ijms19092585
关键词
pulmonary arterial hypertension; transforming growth factor beta; bone morphogenetic protein; signaling; pathophysiology; treatment
资金
- Dutch Lung Foundation [5.2.17.198JO]
- Netherlands CardioVascular Research Initiative: The Dutch Heart Foundation
- Dutch Federation of University Medical Centers
- Netherlands Organisation for Health Research and Development
- Royal Netherlands Academy of Sciences [2012-08]
Knowledge pertaining to the involvement of transforming growth factor beta (TGF-beta) 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-beta signaling in PAH. This review gives a state of the art overview of disturbed signaling through the receptors of the TGF-beta 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.
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