The various roles of nephrin at the slit diaphram and as a modulator of prosurvival signaling in podocytes are well-documented (23–28) and will not be detailed in this review, however, it is clear that disease processes that impair podocyte nephrin expression, and other slit diaphragm components, result in podocyte dysfunction and drive the development of proteinuria and the FSGS lesion (29). The gene discussed is NPHS1; the disease is focal segmental glomerulosclerosis.