Heart valve development: regulatory networks in development and disease.
Heart valve development: regulatory networks in development and disease.
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DOI：
10.1161/circresaha.109.201566
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发表时间：
2009-08-28
影响因子：
20.1
通讯作者：
Yutzey KE
中科院分区：
文献类型：
--
作者：
Combs MD;Yutzey KE
近年来，在控制正常和异常心脏瓣膜发育的调控途径的定义方面取得了重大进展。本文综述了瓣膜祖细胞早期发育和正常瓣膜结构和功能建立的细胞和分子机制。在脊椎动物瓣膜发生过程中，由多种信号通路、转录因子和下游结构基因组成的调控等级是保守的。心内膜垫形成、瓣膜祖细胞增殖、瓣膜细胞系发育以及分层瓣膜小叶中细胞外基质（ECM）室的建立需要复杂的交叉调控途径。越来越多的证据表明，控制正常瓣膜发育的调节机制也有助于人类瓣膜病理。此外，先天性瓣膜畸形在晚期更换的病变瓣膜中占主导地位。了解瓣膜发育机制对先天性和成人瓣膜疾病的诊断和治疗具有重要意义。
In recent years, significant advances have been made in the definition of regulatory pathways that control normal and abnormal cardiac valve development. Here, we review the cellular and molecular mechanisms underlying the early development of valve progenitors and establishment of normal valve structure and function. Regulatory hierarchies consisting of a variety of signaling pathways, transcription factors, and downstream structural genes are conserved during vertebrate valvulogenesis. Complex intersecting regulatory pathways are required for endocardial cushion formation, valve progenitor cell proliferation, valve cell lineage development, and establishment of extracellular matrix (ECM) compartments in the stratified valve leaflets. There is increasing evidence that the regulatory mechanisms governing normal valve development also contribute to human valve pathology. In addition, congenital valve malformations are predominant among diseased valves replaced late in life. The understanding of valve developmental mechanisms has important implications in the diagnosis and management of congenital and adult valve disease.