Role of neural crest-derived cells in the pathogenesis of pulmonary hypertension and new mechanisms of en endothelin receptor antagonist.

P0-Cre小鼠，体内可追踪到P0-Cre阳性神经嵴源性细胞，暴露于慢性低压缺氧3周。在这个模型中，我们可以证明肺动脉高压的典型表现，包括肺动脉压升高、右心室肥厚和小的肺动脉周围肌肉化。在这个模型中，我们可以用免疫组织化学方法证明一些肺血管细胞是神经嵴来源的。在这个模型中，我们可以证明内皮素受体拮抗剂可以改善肺动脉压、右心室肥厚和小肺动脉的肌肉化。我们目前正在研究肺血管中神经嵴来源细胞的动力学。

P0-Cre mice, in which P0-Cre positive neural crest-derived cells can be traced in vivo, were exposed to chronic hypobaric hypoxia for 3 weeks. In this model, we could demonstrated the typical findings consistent with pulmonary arterial hypertension, including increased pulmonary artery pressure, right ventricular hypertrophy, and muscularization of small perioheral pulmonary arteries. In this model, we could demonstrated immunohistochemically that some pulmonary vascular cells are neural crest-derived. In this model, we could show that an endothelin receptor antagonist ameliorated pulmonary artery pressure, right ventricular hypertrophy, and muscularization of small pulmonary arteries. We are currently investigating the kinetics of neural crest-derived cells in pulmonary vessels.