Rosenquist T H, Kirby M L, van Mierop L H
Department of Anatomy, Medical College of Georgia, Augusta 30912-2000.
Circulation. 1989 Nov;80(5):1469-75. doi: 10.1161/01.cir.80.5.1469.
Cells from the cardiac neural crest are essential for the normal development of both the heart and the great vessels. If cardiac neural crest is ablated surgically from Hamburger-Hamilton stage 9 chicken embryos, they will develop anomalies of both the heart and great vessels that are similar to anomalies that occur in humans. In the absence of cardiac neural crest, another area of neural ectoderm (nodose placode) provides replacement cells that are less competent than those of the neural crest. In this study, both the cardiac neural crest and the nodose placodes have been surgically ablated. A syndrome of unusual prevalence (47%) and severity was found among the survivors of this surgery, which was characterized by a large undivided aorta that arched dorsally without right or left deviation to become the dorsal aorta. There was no other tributary to the formation of the dorsal aorta. There were no ducti arteriosi, and the pulmonary arteries were both ectopic and hypoplastic. The brachiocephalic arteries were asymmetric and hypoplastic. The association of the aorta with the anlagen of the thyroid and thymus glands, as well as with the inferior ganglion of the vagus nerve, indicated that the solitary surviving aortic arch artery is that of arch III in this syndrome. These results establish a biological limit of the plasticity of the neural ectoderm and give a probable cellular basis for a lethal congenital septal defect.
心脏神经嵴的细胞对于心脏和大血管的正常发育至关重要。如果在汉伯格-汉密尔顿9期鸡胚中通过手术切除心脏神经嵴，它们将发育出心脏和大血管的异常，这些异常与人类发生的异常相似。在没有心脏神经嵴的情况下，神经外胚层的另一个区域（结节原基）会提供替代细胞，但其能力不如神经嵴细胞。在本研究中，心脏神经嵴和结节原基均已通过手术切除。在该手术的幸存者中发现了一种具有异常高患病率（47%）和严重程度的综合征，其特征是一条未分隔的大主动脉背向拱起，无左右偏差，成为背主动脉。背主动脉形成没有其他分支。没有动脉导管，肺动脉异位且发育不全。头臂动脉不对称且发育不全。主动脉与甲状腺和胸腺原基以及迷走神经下神经节的关联表明，在该综合征中，唯一存活的主动脉弓动脉是Ⅲ型主动脉弓。这些结果确定了神经外胚层可塑性的生物学极限，并为致命性先天性间隔缺损提供了可能的细胞基础。
