An essential role of Bmp4 in the atrioventricular septation of the mouse heart

An essential role of Bmp4 in the atrioventricular septation of the mouse heart
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DOI:
10.1101/gad.1124803
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发表时间:
2003-10-01
影响因子:
10.5
通讯作者:
Hogan, BLM
Hogan, BLM
中科院分区:
生物学1区
文献类型:
--
作者:
Jiao, K;Kulessa, H;Hogan, BLM

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在心脏发生过程中,正确的分隔和瓣膜形成取决于心肌和内膜之间的相互作用。通过结合使用亚型骨形态发生蛋白4(Bmp4)等位基因与条件性基因失活,我们在这里确定Bmp4作为一个信号,从心肌直接介导房室间隔。这一过程中的缺陷导致最常见的人类先天性心脏异常之一,房室管缺陷(AVCD)。通过改变心肌中Bmp4表达获得的缺陷谱概括了患者中诊断的AVCD的范围,从而提供了以AVCD为主要缺陷的有用的遗传模型。
Proper septation and valvulogenesis during cardiogenesis depend on interactions between the myocardium and the endocardium. By combining use of a hypomorphic Bone morphogenetic protein 4 (Bmp4) allele with conditional gene inactivation, we here identify Bmp4 as a signal from the myocardium directly mediating atrioventricular septation. Defects in this process cause one of the most common human congenital heart abnormalities, atrioventricular canal defect (AVCD). The spectrum of defects obtained through altering Bmp4 expression in the myocardium recapitulates the range of AVCDs diagnosed in patients, thus providing a useful genetic model with AVCD as the primary defect.