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TGFβ and BMPRII signalling pathways in the pathogenesis of pulmonary arterial hypertension
Drug Discovery Today ( IF 7.4 ) Pub Date : 2018-12-07 , DOI: 10.1016/j.drudis.2018.12.001
Birger Tielemans , Marion Delcroix , Catharina Belge , Rozenn Quarck

Pulmonary arterial hypertension (PAH) is a severe condition characterised by remodelling of precapillary pulmonary arteries sometimes associated with mutations in the bone morphogenetic protein receptor type 2 (BMPR2) gene. Even in the absence of BMPR2 mutations, increased transforming growth factor (TGF)β receptor signalling and decreased BMPRII signalling have been shown to contribute to PAH pathogenesis. In this Keynote, we review the potential mechanisms by which the imbalance of BMP/TGFβ signalling contributes to endothelial dysfunction, vascular remodelling, inflammation and disordered angiogenesis in PAH. Additionally, we highlight how currently used drugs can influence BMP/TGFβ signalling. Finally, we browse the newly developed therapeutic approaches targeting BMPRII and TGFβ signalling pathways by focusing on preclinical studies and clinical trials and put them into perspectives.



中文翻译:

TGFβ和BMPRII信号通路在肺动脉高压发病中的作用

肺动脉高压(PAH)是一种严重疾病,其特征是毛细血管前肺动脉重塑,有时与2型骨形态发生蛋白受体BMPR2)基因的突变有关。即使没有BMPR2突变,增加的转化生长因子(TGF)β受体信号传导和降低的BMPRII信号传导已被证明可导致PAH发病。在本主题演讲中,我们回顾了BMP /TGFβ信号失衡导致PAH中内皮功能障碍,血管重塑,炎症和血管生成紊乱的潜在机制。此外,我们重点介绍了目前使用的药物如何影响BMP /TGFβ信号传导。最后,我们将重点放在临床前研究和临床试验上,浏览针对BMPRII和TGFβ信号通路的新开发治疗方法,并将其纳入观点。

更新日期:2018-12-07
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