Essential roles of Gli3 and sonic hedgehog in pattern formation and developmental anomalies caused by their dysfunction
Essential roles of Gli3 and sonic hedgehog in pattern formation and developmental anomalies caused by their dysfunction
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DOI：
10.1111/j.1741-4520.2006.00114.x
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发表时间：
2006-09-01
影响因子：
1.3
通讯作者：
Motoyama, Jun
中科院分区：
文献类型：
--
作者：
Motoyama, Jun
沿着体轴的图案形成指导功能性组织结构所需的不同类型细胞的比例。分泌的蛋白质音刺猬（Shh）和锌指转录因子Gli 3在大脑和肢体发育过程中的模式形成的关键球员; Shh对Gli 3的拮抗作用可能是至关重要的模式形成。Shh/Gli 3双纯合突变体的最新研究结果表明，在器官发生过程中，两种活性的平衡是生产正常比例的不同细胞类型所必需的。该结论与Shh梯度指导几种不同细胞类型的规格的备择假设形成对比。这里回顾的意见提供了一个新的视角来了解人类出生缺陷的Shh和Gli 3基因突变引起的发病机制。
Pattern formation along the body axis directs the proportion of different types of cells required for functional tissue structures. The secreted protein sonic hedgehog (Shh) and zinc finger transcription factor Gli3 are key players in pattern formation during brain and limb development; the antagonistic action of Shh towards Gli3 may be crucial for pattern formation. Recent findings from Shh/Gli3 double homozygous mutants suggest that a balance of both activities is required for the production of the normal proportion of different cell types during organogenesis. This conclusion contrasts with the alternative hypothesis that a Shh gradient directs the specification of several different cell types. The observations reviewed here offer a new perspective on understanding the pathogenesis of human birth defects caused by mutations of the Shh and Gli3 genes.