The evolutionarily conserved planar cell polarity (PCP) signaling pathway controls tissue polarity within the plane orthogonal to the\napical-basal axis. PCP was originally discovered in Drosophila melanogaster where it is required for the establishment of a uniform\npattern of cell structures and appendages. In vertebrates, including mammals, the PCP pathway has been adapted to control various\nmorphogenetic processes that are critical for tissue and organ development. These include convergent extension (crucial for neural\ntube closure and cochlear duct development) and oriented cell division (needed for tubular elongation), ciliary tilting that enables\ndirectional fluid flow, and other processes. Recently, strong evidence has emerged to implicate the PCP pathway in vertebrate kidney\ndevelopment. In this review, we will describe the experimental data revealing the role of PCP signaling in nephrogenesis and kidney\ndisease.
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