Pdgfrα functions in endothelial-derived cells to regulate neural crest cells and the development of the great arteries.
Pdgfrα functions in endothelial-derived cells to regulate neural crest cells and the development of the great arteries.
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DOI：
10.1242/dmm.029710
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发表时间：
2017-09-01
影响因子：
4.3
通讯作者：
Jain R
中科院分区：
文献类型：
--
作者：
Aghajanian H;Cho YK;Rizer NW;Wang Q;Li L;Degenhardt K;Jain R
起源于胚胎心脏的单一血管，动脉干必须分离并重塑成主动脉和肺动脉，以支持出生后的生命。有缺陷的重塑或分隔导致的异常统称为圆锥锥体缺陷，这与显著的死亡率和发病率相关。多个细胞群必须相互作用来协调流出道重构，而心脏神经嵴在这一过程中显得尤为重要。心脏神经嵴的异常与多种圆锥锥体缺陷的发病机制有关，包括持续性动脉干、双出口右心室和法洛四联症。然而，神经嵴在另一种圆锥锥体异常（大动脉转位）发病机制中的作用尚不清楚。在本报告中，我们证明了Pdgfra在流出道发育过程中内皮细胞及其衍生物中的意想不到的作用。内皮细胞和内皮细胞中Pdgfra的缺失导致双出口右心室和大动脉转位。我们的数据表明，流出道心内膜缓冲层内皮源间质中Pdgfra的缺失会导致发育过程中神经嵴迁移的继发性缺陷。摘要：内皮源性间质中Pdgfrα的缺失导致神经嵴行为缺陷，并与锥体缺损相关，包括大动脉转位。
Originating as a single vessel emerging from the embryonic heart, the truncus arteriosus must septate and remodel into the aorta and pulmonary artery to support postnatal life. Defective remodeling or septation leads to abnormalities collectively known as conotruncal defects, which are associated with significant mortality and morbidity. Multiple populations of cells must interact to coordinate outflow tract remodeling, and the cardiac neural crest has emerged as particularly important during this process. Abnormalities in the cardiac neural crest have been implicated in the pathogenesis of multiple conotruncal defects, including persistent truncus arteriosus, double outlet right ventricle and tetralogy of Fallot. However, the role of the neural crest in the pathogenesis of another conotruncal abnormality, transposition of the great arteries, is less well understood. In this report, we demonstrate an unexpected role of Pdgfra in endothelial cells and their derivatives during outflow tract development. Loss of Pdgfra in endothelium and endothelial-derived cells results in double outlet right ventricle and transposition of the great arteries. Our data suggest that loss of Pdgfra in endothelial-derived mesenchyme in the outflow tract endocardial cushions leads to a secondary defect in neural crest migration during development. Summary: Loss of Pdgfrα in endothelial-derived mesenchyme results in defective neural crest behavior and is associated with conotruncal defects including, surprisingly, transposition of the great arteries.
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DOI：
10.1073/pnas.90.2.532
发表时间：
1993-01-15
影响因子：
11.1
作者：
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通讯作者：
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影响因子：
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影响因子：
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影响因子：
25
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