Hedgehog in the human:: A possible explanation for the VATER association

被引：23

作者：

Arsic, D ^{[1
]}

Qi, BQ ^{[1
]}

Beasley, SW ^{[1
]}

机构：

[1] Christchurch Hosp, Dept Paediat Surg, Christchurch, New Zealand

来源：

JOURNAL OF PAEDIATRICS AND CHILD HEALTH | 2002年 / 38卷 / 02期

关键词：

adriamycin; foregut; Gli genes; notochord; Sonic hedgehog gene; VATER association;

D O I：

10.1046/j.1440-1754.2002.00813.x

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Foregut malformations are relatively common anomalies, occurring in 1 in 2000-5000 live births. The adriamycin-induced rat model of the VATER association has provided a means of studying the morphogenesis of a variety of major congenital structural abnormalities similar to those seen in humans with VATER association. The secreted glycoprotein, Sonic hedgehog (Shh), may act as an endodermal signal that controls gut and lung patterning. Mice with targeted deletion of Shh have foregut defects that are consistent with those produced by administration of adriamycin. It is possible that mutations induced by adriamycin may result from the breakdown of the Shh signalling pathway.

引用

页码：117 / 121

页数：5

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