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分支形态发生的基因调控:从功能缺失表型中获得的经验教训。

Genetic regulation of branching morphogenesis: lessons learned from loss-of-function phenotypes.

作者信息

Hu Ming Chang, Rosenblum Norman D

机构信息

Program in Developmental Biology, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada.

出版信息

Pediatr Res. 2003 Oct;54(4):433-8. doi: 10.1203/01.PDR.0000085170.44226.DB. Epub 2003 Aug 6.

DOI:10.1203/01.PDR.0000085170.44226.DB
PMID:12904600
Abstract

Branching morphogenesis, defined as growth and branching of epithelial tubules during embryogenesis, is a fundamental feature of renal, lung, mammary gland, submandibular gland, and pancreatic morphogenesis in mammals. Disruption of branching morphogenesis has been demonstrated to result in maldevelopment of some of these organs. Genetic studies performed in affected humans and mutant mice have implicated transcription factors, secreted growth factors, and cell surface signaling molecules as critical regulators of branching morphogenesis. These factors function within networks that appear to exert tight control over the number and location of branches. This review summarizes current knowledge regarding the molecular control of branching morphogenesis in vivo with particular emphasis on the genetic contribution to perturbed branching morphogenesis in mice and humans.

摘要

分支形态发生被定义为胚胎发育过程中上皮小管的生长和分支,是哺乳动物肾脏、肺、乳腺、下颌下腺和胰腺形态发生的基本特征。已证明分支形态发生的破坏会导致其中一些器官发育不良。在受影响的人类和突变小鼠中进行的遗传学研究表明,转录因子、分泌型生长因子和细胞表面信号分子是分支形态发生的关键调节因子。这些因子在似乎对分支的数量和位置进行严格控制的网络中发挥作用。本综述总结了目前关于体内分支形态发生分子控制的知识,特别强调了基因对小鼠和人类分支形态发生紊乱的影响。

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