Tout-velu is a Drosophila homologue of the putative tumour suppressor EXT-1 and is needed for Hh diffusion
Tout-velu is a Drosophila homologue of the putative tumour suppressor EXT-1 and is needed for Hh diffusion
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DOI：
10.1038/27932
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发表时间：
1998-07-02
期刊：
影响因子：
64.8
通讯作者：
Perrimon, N
中科院分区：
文献类型：
--
作者：
Bellaiche, Y;The, I;Perrimon, N
Hedgehog（Hh）蛋白在无脊椎动物和脊椎动物发育过程中通过短程和长程信号传导作用于组织模式（1）。Hedgehog能够长距离扩散并发挥其长距离效应的机制尚不清楚。在这里，我们发现了一个新的果蝇基因，名为tout-velu，它是Hedgehog扩散所需的。tout-velu的表征表明，它编码一个完整的膜蛋白，属于EXT基因家族。该家族的成员参与了人类多发性外生骨疣综合征，其影响骨形态发生（2-4）。我们的研究结果，以及先前对印度刺猬在骨形态发生中的作用的描述（5-7），使我们提出多发性外生骨疣综合征与刺猬蛋白的异常扩散有关。这些结果表明，存在一个新的保守机制所需的扩散刺猬。
Hedgehog (Hh) proteins act through both short-range and long-range signalling to pattern tissues during invertebrate and vertebrate development(1). The mechanisms allowing Hedgehog to diffuse over a long distance and to exert its long-range effects are not understood. Here we identify a new Drosophila gene, named tout-velu, that is required for diffusion of Hedgehog. Characterization of tout-velu shows that it encodes an integral membrane protein that belongs to the EXT gene family. Members of this family are involved in the human multiple exostoses syndrome, which affects bone morphogenesis(2-4). Our results, together with the previous characterization of the role of Indian Hedgehog in bone morphogenesis(5-7), lead us to propose that the multiple exostoses syndrome is associated with abnormal diffusion of Hedgehog proteins. These results show the existence of a new conserved mechanism required for diffusion of Hedgehog.