Cyclopia and defective axial patterning in mice lacking Sonic hedgehog gene function
Cyclopia and defective axial patterning in mice lacking Sonic hedgehog gene function
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DOI：
10.1038/383407a0
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发表时间：
1996-10-03
期刊：
影响因子：
64.8
通讯作者：
Beachy, PA
中科院分区：
文献类型：
--
作者：
Chiang, C;Ying, LTT;Beachy, PA
对小鼠的靶向基因破坏表明，Sonic hedgehog(Shh)基因在脊椎动物胚胎组织的模式形成中发挥关键作用，包括大脑和脊髓、中轴骨骼和四肢。早期的缺陷是建立或维持中线结构，如脊索和底板，后来的缺陷包括远端肢体结构的缺失，睫状眼，神经管内腹侧细胞类型的缺失，以及脊柱和大部分肋骨的缺失。所有组织中的缺陷都超出了Shh转录的正常位置，证实了Shh蛋白作为细胞外信号的作用，这是几种脊椎动物模式中心组织组织特性所必需的。
Targeted gene disruption in the mouse shows that the Sonic hedgehog (Shh) gene plays a critical role in patterning of vertebrate embryonic tissues, including the brain and spinal cord, the axial skeleton and the limbs. Early defects are observed in the establishment or maintenance of midline structures, such as the notochord and the floorplate, and later defects include absence of distal limb structures, cyclopia, absence of ventral cell types within the neural tube, and absence of the spinal column and most of the ribs. Defects in all tissues extend beyond the normal sites of Shh transcription, confirming the proposed role of Shh proteins as an extracellular signal required for the tissue-organizing properties of several vertebrate patterning centres.