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Updated: May 22, 2026

Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
Published on: February 21, 2016
Planar cell polarity genes, Celsr1-3, in neural development
Jia Feng1, Qi Han, Libing Zhou
1Joint Laboratory for Brain Function and Health, Jinan University and The University of Hong Kong, Medical School of Jinan University, Guangzhou 510632, China.
Flamingo proteins are crucial for cell polarity and neuronal development in flies and mammals. Mammalian Celsr1-3 genes cooperate in neural development, impacting axon guidance and cell migration.
Area of Science:
- Cell Biology
- Developmental Biology
- Neuroscience
Background:
- Flamingo proteins belong to a unique cadherin subfamily with distinct structural features compared to classic cadherins.
- Drosophila Flamingo plays key roles in epithelial polarity and neuronal morphogenesis.
- Mammalian orthologs (Celsr1-3) are expressed in the nervous system and are implicated in neural development.
Purpose of the Study:
- To investigate the roles and interactions of mammalian Flamingo orthologs (Celsr1-3) in neural development.
Main Methods:
- Comparative analysis of Flamingo/Celsr protein structure.
- Examination of Celsr1-3 gene knockout mouse phenotypes.
- Assessment of Celsr1-3 cooperative interactions in neural development.
Main Results:
- Mammalian Celsr1-3 proteins are involved in critical neural processes including axon guidance, neuronal migration, and cilium polarity.
- Single-gene knockout mice for Celsr1-3 display varied phenotypes.
- Evidence suggests cooperative interactions among Celsr1-3 genes.
Conclusions:
- Mammalian Celsr proteins are essential for multiple aspects of neural development.
- Cooperative functions among Celsr1-3 genes are important for normal neural development.
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