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左右不对称与先天性心脏缺陷:探寻脊椎动物左右轴确定的核心问题。

Left-right asymmetry and congenital cardiac defects: getting to the heart of the matter in vertebrate left-right axis determination.

作者信息

Ramsdell Ann F

机构信息

Department of Cell and Developmental Biology and Anatomy, School of Medicine and Program in Women's Studies, College of Arts and Sciences, University of South Carolina, Columbia, SC 29208, USA.

出版信息

Dev Biol. 2005 Dec 1;288(1):1-20. doi: 10.1016/j.ydbio.2005.07.038. Epub 2005 Nov 11.

DOI:10.1016/j.ydbio.2005.07.038
PMID:16289136
Abstract

Cellular and molecular left-right differences that are present in the mesodermal heart fields suggest that the heart is lateralized from its inception. Left-right asymmetry persists as the heart fields coalesce to form the primary heart tube, and overt, morphological asymmetry first becomes evident when the heart tube undergoes looping morphogenesis. Thereafter, chamber formation, differentiation of the inflow and outflow tracts, and position of the heart relative to the midline are additional features of heart development that exhibit left-right differences. Observations made in human clinical studies and in animal models of laterality disease suggest that all of these features of cardiac development are influenced by the embryonic left-right body axis. When errors in left-right axis determination happen, they almost always are associated with complex congenital heart malformations. The purpose of this review is to highlight what is presently known about cardiac development and upstream processes of left-right axis determination, and to consider how perturbation of the left-right body plan might ultimately result in particular types of congenital heart defects.

摘要

中胚层心脏区域中存在的细胞和分子左右差异表明,心脏从一开始就具有左右不对称性。当心脏区域合并形成原始心管时,左右不对称性持续存在,而当心管经历环状形态发生时,明显的形态不对称首先变得明显。此后,腔室形成、流入和流出道的分化以及心脏相对于中线的位置是心脏发育的其他特征,这些特征表现出左右差异。在人类临床研究和侧位疾病动物模型中的观察表明,心脏发育的所有这些特征都受胚胎左右体轴的影响。当左右轴确定出现错误时,它们几乎总是与复杂的先天性心脏畸形相关。本综述的目的是强调目前已知的心脏发育和左右轴确定的上游过程,并探讨左右身体计划的扰动如何最终导致特定类型的先天性心脏缺陷。

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