McGill University, Montreal, Quebec, Canada.
McGill University Health Center Research Institute, Montreal, Quebec, Canada.
Nat Rev Nephrol. 2021 Jun;17(6):369-385. doi: 10.1038/s41581-021-00395-6. Epub 2021 Feb 5.
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.
平面细胞极性（PCP）是指组织平面中细胞的协调定向。PCP 最初在黑腹果蝇中被发现并研究，现在已广泛存在于脊椎动物中，在脊椎动物中它与器官发生有关。已经确定了特定的 PCP 基因集。这些基因编码的蛋白质在组织平面内的细胞的相对侧不对称分布，并指导许多过程，包括细胞形状和极性的变化、细胞的集体运动或细胞附属物的均匀分布。这些过程的一个共同特征是它们通常涉及肌动球蛋白的重排。PCP 基因的突变可导致许多动物的器官畸形，包括人类。在过去的十年中，有强有力的证据表明 PCP 途径在肾脏发育中起作用，包括输尿管芽的生长和分支形态发生以及足细胞的发育。PCP 信号的缺陷与先天性肾和泌尿道异常的发育性肾脏疾病的发病机制有关。了解 PCP 的起源、分子成分和细胞靶点，为了解 PCP 分子在正常肾脏发育中的参与以及 PCP 成分的功能障碍如何导致肾脏疾病提供了线索。
