INSERM, Center for Interdisciplinary Research in Biology, UMR CNRS 7241/INSERM U1050, Group Pathological Angiogenesis and Vessel Normalization, Collège de France Paris, France.
Front Genet. 2015 Feb 13;6:37. doi: 10.3389/fgene.2015.00037. eCollection 2015.
Defective paracrine Transforming Growth Factor-β (TGF-β) signaling between endothelial cells and the neighboring mural cells have been thought to lead to the development of vascular lesions that are characteristic of Hereditary Hemorrhagic Telangiectasia (HHT). This review highlights recent progress in our understanding of TGF-β signaling in mural cell recruitment and vessel stabilization and how perturbed TGF-β signaling might contribute to defective endothelial-mural cell interaction affecting vessel functionalities. Our recent findings have provided exciting insights into the role of thalidomide, a drug that reduces both the frequency and the duration of epistaxis in individuals with HHT by targeting mural cells. These advances provide opportunities for the development of new therapies for vascular malformations.
内皮细胞与相邻的壁细胞之间旁分泌转化生长因子-β（TGF-β）信号的缺陷被认为导致了遗传性出血性毛细血管扩张症（HHT）特征性的血管病变的发展。这篇综述强调了我们最近在理解 TGF-β信号在招募壁细胞和稳定血管方面的进展，以及失调的 TGF-β信号如何导致影响血管功能的内皮-壁细胞相互作用缺陷。我们最近的发现为沙利度胺的作用提供了令人兴奋的见解，沙利度胺是一种通过靶向壁细胞来减少 HHT 患者鼻出血的频率和持续时间的药物。这些进展为血管畸形的新疗法的发展提供了机会。
