Loss of BMPR-II promotes oxidative stress and inflammation in pulmonary arterial hypertension

肺动脉高压是一种破坏性的疾病，通常影响年轻女性，在确诊后三年内不经治疗就会死亡。新的治疗方法改善了症状，但总体前景依然黯淡。在一种名为骨形态发生蛋白II型受体(BMPR-II)的受体的基因突变被确认之前，这种疾病的原因仍然是一个谜。对这些突变的识别为这种疾病的根本原因提供了第一个真正的洞察力。在莫雷尔教授的实验室里，我们正试图建立BMPR-II突变与肺血管狭窄之间的联系，肺血管狭窄是肺动脉高压的特征。一个潜在的重要观察结果是，BMPR-II受体功能的降低使细胞更容易受到炎症的影响。由于已知血管中的炎症是导致肺动脉高压的原因，这项研究可能会在遗传缺陷和疾病进展之间提供一种新的联系。如果我们能够理解BMPR-II突变引起炎症的机制，我们可能能够阻止这一过程。这样就有可能为这种疾病开发新的治疗方法，例如，使用已经在开发中的针对其他疾病的抗炎方法。

Pulmonary arterial hypertension is a devastating condition, typically affecting young women, which leads to death within three years of diagnosis without treatment. New treatments have improved symptoms but the overall outlook remains poor. The cause of this disease remained a mystery until the identification of genetic mutations in a receptor known as bone morphogenetic protein type II receptor, or BMPR-II. The identification of these mutations has provided the first real insight into the underlying cause of this condition. In Professor Morrell‘s laboratory we are trying to establish the connection between a mutation in BMPR-II and the narrowing of blood vessels in the lung, which characterise pulmonary arterial hypertension. A potentially important observation is that reduced function of the BMPR-II receptor makes cells more susceptible to inflammation. Since inflammation in blood vessels is known to contribute to pulmonary hypertension, this research may provide a novel link between the genetic defect and the progression of disease. If we can understand the mechanism by which BMPR-II mutation causes inflammation we may be able to block this process. It would then be possible to develop new treatments for this condition using, for example, anti-inflammatory approaches that are already under development for other diseases.