TY - JOUR A2 - Fischbach, Michel AU - Gigante, Maddalena AU - Piemontese, Matteo AU - Gesualdo, Loreto AU - Iolascon, Achille AU - Aucella, Filippo PY - 2011 DA - 2011/09/06 TI - Molecular and Genetic Basis of Inherited Nephrotic Syndrome SP - 792195 VL - 2011 AB - Nephrotic syndrome is an heterogeneous disease characterized by increased permeability of the glomerular filtration barrier for macromolecules. Podocytes, the visceral epithelial cells of glomerulus, play critical role in ultrafiltration of plasma and are involved in a wide number of inherited and acquired glomerular diseases. The identification of mutations in nephrin and other podocyte genes as causes of genetic forms of nephrotic syndrome has revealed new important aspects of the pathogenesis of proteinuric kidney diseases and expanded our knowledge of the glomerular biology. Moreover, a novel concept of a highly dynamic slit diaphragm proteins is emerging. The most significant discoveries in our understanding of the structure and function of the glomerular filtration barrier are reviewed in this paper. SN - 2090-214X UR - https://doi.org/10.4061/2011/792195 DO - 10.4061/2011/792195 JF - International Journal of Nephrology PB - SAGE-Hindawi Access to Research KW - ER -