Inherited Podocyte Diseases

Podocytes are present in the glomerular capillary wall and they are the target cells injured in many diseases. In the past few years genetic studies performed especially in the familial nephrotic syndrome cases have shed light on the recognition of the structure of the glomerular filtration barrier and inherited podocyte disease. Mutations of the podocyte proteinslike nephrin, podocin and α-actinin have been shown to be responsible for the hereditary an familial nephrotic sydromes. Detection of mutations in congenital nephrotic syndrome of the Finnish type, autosomal recessive steroid resistant nephrotic syndrome and autosomal dominant focal segmental glomerulosclerosis lead to improvement in the diagnosis and treatment of these patients. The relevant studies are being carried out more rapidly in accordance with the advances in genetic sciences.