Background: Nephrotic syndrome (NS) is pathological condition characterized by heavy proteinuria. Our study\r\ninvestigates hypothesis that change in cell proliferation of proximal tubules influences primary cilia structure and\r\nfunction and promotes cystogenesis in congenital nephrotic syndrome of the Finnish type (CNF) and focal\r\nsegmental glomerulosclerosis (FSGS).\r\nMethods: CNF kidneys were analyzed genetically. Proliferation (Ki-67), apoptosis (caspase-3), and primary cilia\r\n(a-tubulin) length and structure were analyzed immunohistochemically and ultrastructurally in healthy, CNF and\r\nFSGS kidneys. Cyst diameters were measured and correlated with proliferation index.\r\nResults: Proximal tubules cells of healthy kidneys did not proliferate. In nephrotic kidneys, tubules with apparently\r\nnormal diameter covered by cuboidal/columnar epithelium (PTNC) contained 81.54% of proliferating cells in CNF\r\nand 36.18% in FSGS, while cysts covered with columnar epithelium (CC) contained 37.52% of proliferating cells in\r\nCNF and 45.23% in FSGS. The largest cysts, covered with squamous epithelium (CS) had 11.54% of proliferating cells\r\nin CNF and 13.76% in FSGS. Increase in cysts diameter correlated with changes in proliferation index, tubular cells\r\nshape, primary cilia formation and appearance of apoptotic cells.\r\nConclusions: We present a novel histopathological data on the structure and possible changes in function of\r\ntubular cell in NS kidneys during cystogenesis. We suggest existence of common principles of cystogenesis in CNF\r\nand FSGS kidneys, including serious disturbances of tubular cells proliferation and apoptosis, and faulty primary cilia\r\nsignaling leading to deterioration of proteinuria in NS kidneys.
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