Planar Cell Polarity Effector Gene Fuzzy Regulates Cilia Formation and Hedgehog Signal Transduction in Mouse
Planar Cell Polarity Effector Gene Fuzzy Regulates Cilia Formation and Hedgehog Signal Transduction in Mouse
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DOI：
10.1002/dvdy.22130
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发表时间：
2009-12-01
影响因子：
2.5
通讯作者：
Liu, Aimin
中科院分区：
文献类型：
--
作者：
Heydeck, Westley;Zeng, Huiqing;Liu, Aimin
精确的平面细胞极性(PCP)对于动物多器官系统的发育至关重要。一组核心-PCP蛋白被认为在哺乳动物的汇聚延伸和其他PCP相关过程中发挥关键作用。然而，另一类PCP调节蛋白-PCP效应蛋白的功能尚未完全研究。在这项研究中，报告了PCP效应基因Fuz(Fuz)小鼠突变体的产生和特性。Fuz纯合子突变体在胚胎上是致命的，具有多种缺陷，包括神经管缺陷、脊髓背侧/腹侧异常模式，以及肢体芽的前后模式缺陷。Fuz突变体还表现出Hedgehog(HH)信号的异常和Gli3蛋白降解过程的低效。最后，在Fuz纯合子突变体中发现纤毛显著减少。综上所述，FUZ在哺乳动物纤毛形成、HH信号转导和胚胎发育中起着重要作用。发展动力学238：3035-3042,2009。(C)2009年Wiley-Liss，Inc.
Precise planar cell polarity (PCP) is critical for the development of multiple organ systems in animals. A group of core-PCP proteins are recognized to play crucial roles in convergent extension and other PCP-related processes in mammals. However, the functions of another group of PCP-regulating proteins, the PCP-effector proteins, are yet to be fully studied. In this study, the generation and characterization of a mouse mutant for the PCP effector gene Fuzzy (Fuz) is reported. Fuz homozygous mutants are embryonically lethal, with multiple defects including neural tube defects, abnormal dorsal/ventral patterning of the spinal cord, and defective anterior/posterior patterning of the limb buds. Fuz mutants also exhibit abnormal Hedgehog (Hh) signaling and inefficient proteolytic processing of Gli3. Finally, a significant decrease in cilia was found in Fuz homozygous mutants. In conclusion, Fuz plays an important role in cilia formation, Hh signal transduction, and embryonic development in mammals. Developmental Dynamics 238:3035-3042, 2009. (C) 2009 Wiley-Liss, Inc.