Planar cell polarity effector gene Fuzzy regulates cilia formation and hedgehog signal transduction in mouse

Westley Heydeck, Huiqing Zeng, Aimin Liu

Research output: Contribution to journalArticle

64 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)3035-3042
Number of pages8
JournalDevelopmental Dynamics
Volume238
Issue number12
DOIs
StatePublished - Dec 1 2009

All Science Journal Classification (ASJC) codes

  • Developmental Biology

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