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全身多毛蛋白是假定的肿瘤抑制因子EXT-1的果蝇同源物,是Hh扩散所必需的。

Tout-velu is a Drosophila homologue of the putative tumour suppressor EXT-1 and is needed for Hh diffusion.

作者信息

Bellaiche Y, The I, Perrimon N

机构信息

Department of Genetics, Harvard Medical School, Boston, Massachusetts 02115, USA.

出版信息

Nature. 1998 Jul 2;394(6688):85-8. doi: 10.1038/27932.

Abstract

Hedgehog (Hh) proteins act through both short-range and long-range signalling to pattern tissues during invertebrate and vertebrate development. 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. Our results, together with the previous characterization of the role of Indian Hedgehog in bone morphogenesis, 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.

摘要

刺猬(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来调控组织形态。目前尚不清楚刺猬蛋白能够远距离扩散并发挥其长程效应的机制。在此,我们鉴定出一个新的果蝇基因,命名为tout-velu,它是刺猬蛋白扩散所必需的。对tout-velu的特性分析表明,它编码一种属于EXT基因家族的整合膜蛋白。该家族成员与人类多发性外生骨疣综合征有关,该综合征会影响骨骼形态发生。我们的研究结果,连同之前对印度刺猬蛋白在骨骼形态发生中作用的特性分析,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种新的刺猬蛋白扩散所需的保守机制。

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