Heart defects in connexin43-deficient mice

被引:121
作者
Ya, J
Erdtsieck-Ernste, EBHW
de Boer, PAJ
van Kempen, MJA
Jongsma, H
Gros, D
Moorman, AFM
Lamers, WH
机构
[1] Univ Amsterdam, Acad Med Ctr, Dept Anat & Embryol, NL-1105 AZ Amsterdam, Netherlands
[2] Univ Utrecht, Dept Med Physiol, NL-3508 TC Utrecht, Netherlands
[3] Fac Sci Luminy, Inst Dev Biol, F-13288 Marseille, France
关键词
connexin; cardiac malformation; cardiac development;
D O I
10.1161/01.RES.82.3.360
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Cardiac malformation in connexin43 (CX43)-disrupted mice is restricted to the junction between right ventricle and outflow tract, even though CX43 is also expressed abundantly elsewhere. We analyzed cardiac morphogenesis in immunohistochemically and hybridohistochemically stained and three-dimensionally reconstructed serial sections of CX43-deficient embryos between embryonic day (ED) 10 and birth. The establishment of the D configuration in the ascending loop of CX43-deficient hearts is markedly retarded, so that the right ventricle retains a craniomedial position and is connected with the outflow tract by a more acute bend in ED10 and ED11 embryos. Because of the subsequent growth of the right ventricle, this condition usually evolves into a D loop, but when it persists, a "crisscross" configuration develops, with the atrioventricular cushions rotated 90 degrees, a horizontal muscular ventricular septum, and a parallel course of the endocardial ridges of the outflow tract. After ED12, large intertrabecular pouches develop at the ventricular side of both shelflike myocardial structures that support the endocardial ridges of the outflow tract, ie, at the location that was earlier characterized by the acute bend between the right ventricle and the outflow tract and that subsequently develops into the anterosuperior leaflet of the tricuspid valve. Retarded development of the D configuration in the ascending loop of the embryonic heart predisposes the myocardium at the junction of the right ventricle and outflow tract to excessive development-of intertrabecular pouches during subsequent development.
引用
收藏
页码:360 / 366
页数:7
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