心脏瓣膜结构及其功能对心脏发育和心脏疾病的影响

The structure and function of the heart valves affect the cardiac development and heart diseases

发育成熟的心脏瓣膜是由一层内皮细胞包裹的高度有序的细胞外基质和瓣膜间质细胞组成。心脏瓣膜的细胞外基质根据对瓣叶和支撑结构的不同生物学性能可以分为三类:富弹性蛋白层、富粘蛋白层和富胶原蛋白层。信号转导通路在原始瓣膜形成及瓣膜结构功能的持续维持上起着至关重要的作用。动物模型为研究瓣膜发育及其相关疾病过程提供了很重要的支持。瓣膜疾病是一个具有重要意义的公共卫生健康问题,越来越多的证据显示畸形发育是其发病机制的一个基础。未来需要更多更深层次的研究来探索调节瓣膜发育各个机制途径的相互作用,从而为新的疗法的诞生奠定基础。

The mature heart valves are made up of highly organized extracellular matrix （ECM） and valve interstitial cells （VICs） surrounded by an endothelial cell layer. The ECM of the valves is stratified into elastin-, proteoglycan-, and collagen-rich layers that confer distinct biomechanical properties to the leaflets and supporting structures. Signaling pathways have critical functions in primary valvulogenesis as well as the maintenance of valve structure and function over time. Animal models provide powerful tools to study valve development and disease processes. Valve disease is a significant public health problem, and increasing evidence implicates aberrant developmental mechanisms underlying pathogenesis. Further studies are necessary to determine regulatory pathway interactions underlying valve pathogenesis in order to generate new avenues for novel therapeutics.