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Cardiac Neural Crest Cells: Their Rhombomeric Specification, Migration, and Association with Heart and Great Vessel Anomalies.
Cellular and Molecular Neurobiology ( IF 3.6 ) Pub Date : 2020-05-13 , DOI: 10.1007/s10571-020-00863-w
Olivier Schussler 1, 2 , Lara Gharibeh 3 , Parmeseeven Mootoosamy 1 , Nicolas Murith 1 , Vannary Tien 4 , Anne-Laure Rougemont 5 , Tornike Sologashvili 1 , Erik Suuronen 6, 7 , Yves Lecarpentier 8 , Marc Ruel 7
Affiliation  

Outflow tract abnormalities are the most frequent congenital heart defects. These are due to the absence or dysfunction of the two main cell types, i.e., neural crest cells and secondary heart field cells that migrate in opposite directions at the same stage of development. These cells directly govern aortic arch patterning and development, ascending aorta dilatation, semi-valvular and coronary artery development, aortopulmonary septation abnormalities, persistence of the ductus arteriosus, trunk and proximal pulmonary arteries, sub-valvular conal ventricular septal/rotational defects, and non-compaction of the left ventricle. In some cases, depending on the functional defects of these cells, additional malformations are found in the expected spatial migratory area of the cells, namely in the pharyngeal arch derivatives and cervico-facial structures. Associated non-cardiovascular anomalies are often underestimated, since the multipotency and functional alteration of these cells can result in the modification of multiple neural, epidermal, and cervical structures at different levels. In most cases, patients do not display the full phenotype of abnormalities, but congenital cardiac defects involving the ventricular outflow tract, ascending aorta, aortic arch and supra-aortic trunks should be considered as markers for possible impaired function of these cells. Neural crest cells should not be considered as a unique cell population but on the basis of their cervical rhombomere origins R3-R5 or R6-R7-R8 and specific migration patterns: R3-R4 towards arch II, R5-R6 arch III and R7-R8 arch IV and VI. A better understanding of their development may lead to the discovery of unknown associated abnormalities, thereby enabling potential improvements to be made to the therapeutic approach.

中文翻译:

心脏神经嵴细胞:它们的菱形特征、迁移以及与心脏和大血管异常的关联。

流出道异常是最常见的先天性心脏缺陷。这是由于两种主要细胞类型的缺失或功能障碍,即在同一发育阶段向相反方向迁移的神经嵴细胞和次生心场细胞。这些细胞直接控制主动脉弓的形成和发育、升主动脉扩张、半瓣膜和冠状动脉发育、主肺分隔异常、动脉导管的持续存在、主干和近端肺动脉、瓣下锥状室间隔/旋转缺陷和非- 左心室的压实。在某些情况下,根据这些细胞的功能缺陷,在细胞的预期空间迁移区域中会发现额外的畸形,即在咽弓衍生物和宫颈面部结构中。相关的非心血管异常通常被低估,因为这些细胞的多能性和功能改变会导致不同水平的多个神经、表皮和宫颈结构的改变。在大多数情况下,患者不会表现出完整的异常表型,但涉及心室流出道、升主动脉、主动脉弓和主动脉上干的先天性心脏缺陷应被视为这些细胞功能可能受损的标志。神经嵴细胞不应被视为独特的细胞群,而是基于它们的颈部菱形起源 R3-R5 或 R6-R7-R8 和特定的迁移模式:R3-R4 向弓 II、R5-R6 弓 III 和 R7- R8 拱形 IV 和 VI。
更新日期:2020-05-13
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