Mice with an anterior cleft of the palate survive neonatal lethality
Mice with an anterior cleft of the palate survive neonatal lethality
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DOI：
10.1002/dvdy.21534
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发表时间：
2008-05-01
影响因子：
2.5
通讯作者：
Chen, YiPing
中科院分区：
文献类型：
--
作者：
Gu, Shuping;Wei, Na;Chen, YiPing
许多基因在发育中的第二腭中以特定区域的方式起作用。我们之前的研究表明，缺乏shox2的胚胎在妊娠中期死亡，并出现前裂表型。在这里，我们发现携带腭间质Shox2条件失活的小鼠在胚胎和新生儿死亡中存活，但发展为消瘦综合征。表型分析表明，第二腭在前端延迟关闭，导致初级和次级腭融合失败。与Shox2在骨骼形成中的作用一致，Shox2失活导致硬腭骨形成显著减少，可能是由于Runx2和Osterix的下调。我们的结论是，次腭架能够相互融合，但不能与初级腭融合，发育迟缓。携带前裂的小鼠可以在新生儿死亡后存活下来。
Many genes are known to function in a region-specific manner in the developing secondary palate. We have previously shown that Shox2-deficient embryos die at mid-gestation stage and develop an anterior clefting phenotype. Here, we show that mice carrying a conditional inactivation of Shox2 in the palatal mesenchyme survive the embryonic and neonatal lethality, but develop a wasting syndrome. Phenotypic analyses indicate a delayed closure of the secondary palate at the anterior end, leading to a failed fusion of the primary and secondary palates. Consistent with a role proposed for Shox2 in skeletogenesis, Shox2 inactivation causes a significantly reduced bone formation in the hard palate, probably due to a down-regulation of Runx2 and Osterix. We conclude that the secondary palatal shelves are capable of fusion with each other, but fail to fuse with the primary palate in a developmentally delayed manner. Mice carrying an anterior cleft can survive neonatal lethality.