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Updated: Jun 20, 2026
07:07
Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
Published on: February 21, 2016
Planar cell polarity and the kidney
1Samuel Lunenfeld Research Institute, Toronto, Ontario, Canada. mcneill@mshri.on.ca
Journal of the American Society of Nephrology : JASN
|September 19, 2009
Summary
Planar cell polarity (PCP) is crucial for tissue organization and mammalian development. Disruptions in PCP signaling are linked to kidney development defects and cystic kidney disease in mouse models.
Area of Science:
- Developmental Biology
- Cell Biology
- Genetics
Background:
- Planar cell polarity (PCP) is a fundamental mechanism for tissue spatial organization, conserved across species from Drosophila melanogaster to mammals.
- PCP signaling is essential for various aspects of mammalian development, including organogenesis.
- Emerging evidence suggests a role for PCP in kidney development.
Purpose of the Study:
- To review the current understanding of Planar Cell Polarity (PCP) signaling in both invertebrate and vertebrate models.
- To explore the potential link between defects in PCP signaling and the pathogenesis of cystic kidney disease.
- To discuss how disruptions in PCP may contribute to kidney cyst formation.
Main Methods:
- Literature review of existing research on PCP signaling pathways.
- Analysis of data from invertebrate (Drosophila) and vertebrate model systems.
- Examination of studies investigating PCP in kidney development and disease models.
Main Results:
- PCP establishes tissue polarity and orients cell behaviors like division and extension.
- Defects in PCP signaling are associated with abnormal kidney development in mouse models.
- Loss of oriented cell division and convergent extension downstream of faulty PCP signaling contribute to cystic kidney disease.
Conclusions:
- Planar cell polarity is a critical regulator of kidney development.
- Dysfunctional PCP signaling is implicated as a potential cause of cystic kidney disease.
- Further research into PCP mechanisms can illuminate therapeutic strategies for kidney disorders.
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