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Published on: September 1, 2015
Planar cell polarity pathway in kidney development, function and disease
Elena Torban1,2, Sergei Y Sokol3
1McGill University, Montreal, Quebec, Canada. elena.torban@mcgill.ca.
Abstract:
Planar cell polarity (PCP) refers to the coordinated orientation of cells in the tissue plane. Originally discovered and studied in Drosophila melanogaster, PCP is now widely recognized in vertebrates, where it is implicated in organogenesis. Specific sets of PCP genes have been identified. The proteins encoded by these genes become asymmetrically distributed to opposite sides of cells within a tissue plane and guide many processes that include changes in cell shape and polarity, collective cell movements or the uniform distribution of cell appendages. A unifying characteristic of these processes is that they often involve rearrangement of actomyosin. Mutations in PCP genes can cause malformations in organs of many animals, including humans. In the past decade, strong evidence has accumulated for a role of the PCP pathway in kidney development including outgrowth and branching morphogenesis of ureteric bud and podocyte development. Defective PCP signalling has been implicated in the pathogenesis of developmental kidney disorders of the congenital anomalies of the kidney and urinary tract spectrum. Understanding the origins, molecular constituents and cellular targets of PCP provides insights into the involvement of PCP molecules in normal kidney development and how dysfunction of PCP components may lead to kidney disease.
Insights
Planar cell polarity (PCP) guides tissue development by orienting cells. Disruptions in PCP signaling contribute to congenital kidney malformations, highlighting its crucial role in kidney organogenesis.
Area of Science:
- Developmental Biology
- Cell Biology
- Genetics
Background:
- Planar cell polarity (PCP) describes coordinated cell orientation within tissues.
- PCP pathways, involving asymmetric protein distribution, regulate cell shape, movement, and appendage organization.
- Actomyosin rearrangement is a common feature of PCP-guided processes.
Purpose of the Study:
- To review the role of planar cell polarity in vertebrate organogenesis.
- To highlight the specific involvement of PCP in kidney development, including ureteric bud and podocyte formation.
- To explore how PCP dysfunction contributes to congenital kidney disorders.
Main Methods:
- Literature review of PCP research in model organisms and vertebrates.
- Analysis of genetic studies implicating PCP genes in kidney development.
- Examination of molecular mechanisms underlying PCP in kidney organogenesis.
Main Results:
- PCP is essential for normal kidney development, including ureteric bud outgrowth and branching.
- PCP pathway components are critical for proper podocyte development.
- Mutations in PCP genes are linked to congenital anomalies of the kidney and urinary tract (CAKUT).
Conclusions:
- Planar cell polarity is a fundamental pathway in kidney development.
- Dysregulation of PCP signaling is a key factor in the pathogenesis of developmental kidney diseases.
- Further understanding of PCP mechanisms can inform therapeutic strategies for kidney disorders.
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