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首页> 外文期刊>Journal of Cardiovascular Development and Disease >Evidence of Aortopathy in Mice with Haploinsufficiency of Notch1 in Nos3-Null Background
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Evidence of Aortopathy in Mice with Haploinsufficiency of Notch1 in Nos3-Null Background

机译：Nos3-null背景中Notch1单倍缺乏的小鼠主动脉病变的证据。

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摘要

Thoracic aortic aneurysms (TAA) are a significant cause of morbidity and mortality in humans. While the exact etiology is unknown, genetic factors play an important role. Mutations in NOTCH1 have been linked to bicuspid aortic valve (BAV) and aortopathy in humans. The aim of this study was to determine if haploinsufficiency of Notch1 contributes to aortopathy using Notch1+/−; Nos3−/− mice. Echocardiographic analysis of Notch1+/−; Nos3−/− mice reveals effacement of the sinotubular junction and a trend toward dilation of the aortic sinus. Furthermore, examination of the proximal aorta of Notch1+/−; Nos3−/− mice reveals elastic fiber degradation, a trend toward increased matrix metalloproteinase 2 expression, and increased smooth muscle cell apoptosis, features characteristic of aneurysmal disease. Although at a lower penetrance, we also found features consistent with aortopathic changes in Notch1 heterozygote mice and in Nos3-null mice. Our findings implicate a novel role for Notch1 in aortopathy of the proximal aorta.
机译：胸主动脉瘤（TAA）是人类发病和死亡的重要原因。虽然确切的病因尚不清楚，但遗传因素起着重要作用。 NOTCH1的突变与人的二尖瓣主动脉瓣（BAV）和主动脉病变有关。本研究的目的是使用Notch1 +/- 确定Notch1的单倍不足是否导致主动脉病变。 Nos3 -/-小鼠。超声心动图分析Notch1 +/- ; Nos3 -/-小鼠显示出鼻管交界处的消失以及主动脉窦扩张的趋势。此外，检查Notch1 +/- 的近端主动脉; Nos3 -/-小鼠显示弹性纤维降解，基质金属蛋白酶2表达增加的趋势以及平滑肌细胞凋亡的增加，这是动脉瘤疾病的特征。尽管在较低的外显率下，我们还发现了与Notch1杂合子小鼠和Nos3无效小鼠的主动脉病变一致的特征。我们的发现暗示了Notch1在近端主动脉主动脉病变中的新作用。

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