Nephrin is a key molecule in podocytes to maintain normal slit diaphragm structure. Nephin interacts with many other podocyte and slit diaphragm
protein and also mediates important cell signaling pathways in podocytes. Loss of
nephrin during the development leads to the congenital
nephrotic syndrome in children. Reduction of
nephrin expression is often observed in adult
kidney diseases including
diabetic nephropathy and
HIV-associated nephropathy. The critical role of
nephrin has been confirmed by different animal models with
nephrin knockout and knockdown. Recent studies demonstrate that knockdown of
nephrin expression in adult mice aggravates the progression of unilateral
nephrectomy and
Adriamycin-induced
kidney disease. In addition to its critical role in maintaining normal glomerular filtration unit in the kidney,
nephrin is also expressed in other organs. However, the exact role of
nephrin in kidney and extra-renal organs has not been well characterized. Future studies are required to determine whether
nephrin could be developed as a
drug target to treat patients with
kidney disease.