Vandoorne Katrien, Vandsburger Moriel H, Weisinger Karen, Brumfeld Vlad, Hemmings Brian A, Harmelin Alon, Neeman Michal
Biological Regulation, Weizmann Institute of Science Rehovot, Israel ; Biomedical engineering, Eindhoven University of Technology Eindhoven, The Netherlands.
Biological Regulation, Weizmann Institute of Science Rehovot, Israel.
Physiol Rep. 2013 Nov;1(6):e00143. doi: 10.1002/phy2.143. Epub 2013 Nov 7.
Even though congenital heart disease is the most prevalent malformation, little is known about how mutations affect cardiovascular function during development. Akt1 is a crucial intracellular signaling molecule, affecting cell survival, proliferation, and metabolism. The aim of this study was to determine the role of Akt1 on prenatal cardiac development. In utero echocardiography was performed in fetal wild-type, heterozygous, and Akt1-deficient mice. The same fetal hearts were imaged using ex vivo micro-computed tomography (μCT) and histology. Neonatal hearts were imaged by in vivo magnetic resonance imaging. Additional ex vivo neonatal hearts were analyzed using histology and real-time PCR of all three groups. In utero echocardiography revealed abnormal blood flow patterns at the mitral valve and reduced contractile function of Akt1 null fetuses, while ex vivo μCT and histology unraveled structural alterations such as dilated cardiomyopathy and ventricular septum defects in these fetuses. Further histological analysis showed reduced myocardial capillaries and coronary vessels in Akt1 null fetuses. At neonatal age, Akt1-deficient mice exhibited reduced survival with reduced endothelial cell density in the myocardium and attenuated cardiac expression of vascular endothelial growth factor A and collagen Iα1. To conclude, this study revealed a central role of Akt1 in fetal cardiac function and myocardial angiogenesis inducing fetal cardiomyopathy and reduced neonatal survival. This study links a specific physiological phenotype with a defined genotype, namely Akt1 deficiency, in an attempt to pinpoint intrinsic causes of fetal cardiomyopathies.
尽管先天性心脏病是最常见的畸形,但对于突变如何在发育过程中影响心血管功能却知之甚少。Akt1是一种关键的细胞内信号分子,影响细胞存活、增殖和代谢。本研究的目的是确定Akt1在产前心脏发育中的作用。对野生型、杂合子和Akt1缺陷型小鼠胎儿进行宫内超声心动图检查。使用离体微型计算机断层扫描(μCT)和组织学对相同的胎儿心脏进行成像。通过体内磁共振成像对新生小鼠心脏进行成像。对所有三组的离体新生小鼠心脏进行组织学分析和实时PCR分析。宫内超声心动图显示Akt1基因敲除胎儿的二尖瓣血流模式异常,收缩功能降低,而离体μCT和组织学检查发现这些胎儿存在扩张型心肌病和室间隔缺损等结构改变。进一步的组织学分析显示,Akt1基因敲除胎儿的心肌毛细血管和冠状血管减少。在新生期,Akt1缺陷型小鼠的存活率降低,心肌内皮细胞密度降低,血管内皮生长因子A和胶原蛋白Iα1的心脏表达减弱。总之,本研究揭示了Akt1在胎儿心脏功能和心肌血管生成中起核心作用,可导致胎儿心肌病并降低新生儿存活率。本研究将特定的生理表型与特定的基因型(即Akt1缺陷)联系起来,试图找出胎儿心肌病的内在原因。