Planar cell polarity effector gene Fuzzy regulates cilia formation and Hedgehog signal transduction in mouse |
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| Authors: | Westley Heydeck Huiqing Zeng Aimin Liu |
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| Affiliation: | 1. Department of Biology, Eberly College of Science, The Pennsylvania State University, University Park, Pennsylvania;2. W. Heydeck and H. Zeng have contributed equally to this work.;3. Center for Cellular Dynamics, The Programs of Cell and Developmental Biology, Genetics and Neuroscience, Huck Institute of Life Sciences, The Pennsylvania State University, University Park, Pennsylvania |
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| Abstract: | 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. © 2009 Wiley‐Liss, Inc. |
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| Keywords: | Fuzzy cilia planar cell polarity PCP effector hedgehog mouse |
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