Planar cell polarity pathway in kidney development, function and disease

Autor: Sergei Y. Sokol, Elena Torban
Rok vydání: 2021
Předmět:
Zdroj: Nat Rev Nephrol
ISSN: 1759-507X
1759-5061
Popis: Planar cell polarity (PCP) refers to the coordinated orientation of cells in tissue plane. Originally discovered and studied in Drosophila melanogaster, PCP is now widely recognized in vertebrates, where it has been implicated in organogenesis. Specific sets of PCP genes have been identified. The proteins encoded by these genes are organized into a dedicated signaling pathway, become asymmetrically distributed to opposite sides of each cell within a tissue plane and guide many processes that include changes in cell shape and polarity, collective cell movements or uniform distribution of cell appendages. A unifying characteristic of these changes is that they often involve actomyosin rearrangement. Mutations in PCP genes cause congenital malformations of multiple organs in many animals and, importantly, in humans. In the last decade, strong evidence has accumulated for a role of the PCP pathway in kidney development. It has been proposed that defective PCP signaling contributes to polycystic kidney disease and that specific PCP gene mutations lead to Congenital Anomalies of the Kidney and Urinary Tract (CAKUT). In this review, we describe the origins, molecular constituents and cellular targets of PCP, with a special focus on the involvement of PCP molecules in normal kidney development and how their dysfunction leads to kidney disease.
Databáze: OpenAIRE
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