Molecular pathogenesis of pulmonary arterial hypertension
Molecular pathogenesis of pulmonary arterial hypertension
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DOI：
10.1172/jci60658
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发表时间：
2012-12-01
影响因子：
15.9
通讯作者：
Rabinovitch, Marlene
中科院分区：
文献类型：
--
作者：
Rabinovitch, Marlene
最近的临床和实验研究正在重新定义肺动脉高压（PAH）的细胞和分子基础。遗传异常首次被发现与特发性多环芳烃有关，同时我们对细胞信号传导、细胞转化和细胞间相互作用的理解也有了很大的提高；基因表达;microRNA-processing;线粒体和离子通道的功能，有助于解释血管细胞对损伤的异常反应。实验和临床研究。集中于涉及BMPR2信号通路的遗传易感与血管壁不配对的代谢和慢性炎症状态之间的交集和相互作用。这种紊乱的过程最终导致旺盛的增殖反应，阻塞肺动脉（PA）管腔，并使最远端的腺泡内血管闭塞。在这里，我们描述了基于临床前研究的新兴疗法，以解决这些趋同途径。
Recent clinical and experimental studies are redefining the cellular and molecular bases of pulmonary, arterial hypertension (PAH). The genetic abnormalities first identified in association with the idiopathic form of PAH - together with a vast increase in our understanding of cell signaling, cell-transformation, and cell-cell interactions; gene expression; microRNA-processing; and mitochondrial and ion channel function - have helped explain the abnormal response of vascular cells to injury. Experimental and clinical studies now. converge on the intersection and interactions between a genetic predisposition involving the BMPR2 signaling Pathway and an unpaired metabolic and chronic inflammatory state hi the vessel wall The deranged processes' culminate in an exuberant proliferative response that occludes the pulmonary arterial (PA) lumen and obliterates the most distal intraacinar vessels. Here, we describe emerging therapies based on preclinical studies that address these converging pathways.