期刊
CIRCULATION RESEARCH
卷 121, 期 8, 页码 981-999出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1161/CIRCRESAHA.117.309930
关键词
cell differentiation; mutation; signal transduction; transforming growth factors; vascular malformations
资金
- Associazione Italiana per la Ricerca sul Cancro Special Program Molecular Clinical Oncology 5x1000
- European Community (V-EPC [Inherited Dysfunctions of Brain Microcirculation])
- European Community (European Research Council)
- European Community (BtRAIN [Brain Barriers Training] Innovative Training Network)
- Knut and Alice Wallenberg Foundation
- Swedish Research Council
- [TELETHON-GGP14149]
Correct organization of the vascular tree requires the balanced activities of several signaling pathways that regulate tubulogenesis and vascular branching, elongation, and pruning. When this balance is lost, the vessels can be malformed and fragile, and they can lose arteriovenous differentiation. In this review, we concentrate on the transforming growth factor (TGF)-beta/bone morphogenetic protein (BMP) pathway, which is one of the most important and complex signaling systems in vascular development. Inactivation of these pathways can lead to altered vascular organization in the embryo. In addition, many vascular malformations are related to deregulation of TGF-beta/BMP signaling. Here, we focus on two of the most studied vascular malformations that are induced by deregulation of TGF-beta/BMP signaling: hereditary hemorrhagic telangiectasia (HHT) and cerebral cavernous malformation (CCM). The first of these is related to loss-of-function mutation of the TGF-beta/BMP receptor complex and the second to increased signaling sensitivity to TGF-beta/BMP. In this review, we discuss the potential therapeutic targets against these vascular malformations identified so far, as well as their basis in general mechanisms of vascular development and stability.
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