Planar Cell Polarity Effector Gene Fuzzy Regulates Cilia Formation and Hedgehog Signal Transduction in Mouse

被引：70

作者：

Heydeck, Westley ^{[1
]}

Zeng, Huiqing ^{[1
]}

Liu, Aimin ^{[1
,2
,3
]}

机构：

[1] Penn State Univ, Dept Biol, Eberly Coll Sci, University Pk, PA 16802 USA

[2] Penn State Univ, Ctr Cellular Dynam, Program Cell & Dev Biol, University Pk, PA 16802 USA

[3] Penn State Univ, Huck Inst Life Sci, Program Genet & Neurosci, University Pk, PA 16802 USA

来源：

DEVELOPMENTAL DYNAMICS | 2009年 / 238卷 / 12期

关键词：

Fuzzy; cilia; planar cell polarity; PCP effector; hedgehog; mouse; INTRAFLAGELLAR TRANSPORT PROTEINS; REPRESSOR FUNCTIONS; ACTIVATOR; GLI3; MORPHOGENESIS; POLARIZATION; VERTEBRATES; REQUIRES; EMBRYOS; BRAIN;

D O I：

10.1002/dvdy.22130

中图分类号：

R602 [外科病理学、解剖学]; R32 [人体形态学];

学科分类号：

100101 ;

摘要：

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.

引用

页码：3035 / 3042

页数：8

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