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Pediatric Nephrology

Erschienen in:

01.05.2013 | Review

New developments in steroid-resistant nephrotic syndrome

verfasst von: Moin A. Saleem

Erschienen in: Pediatric Nephrology | Ausgabe 5/2013

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Abstract

Nephrotic syndrome is a disorder of the glomerular filtration barrier, a highly specialised tri-layer structure with unique functional properties. Recent advances emanating from the field of molecular genetics have revealed the podocyte as probably the central player in the control of glomerular filtration. More specifically, the cell–cell junction between adjacent podocyte foot processes, namely, the slit diaphragm, has been revealed to be made up of a sophisticated multi-protein complex which dynamically controls foot process architecture via signalling to the actin cytoskeleton. Key genes that have been identified from the study of inherited nephrotic syndromes include those encoding nephrin, podocin, TRPC6 (transient receptor potential canonical channel-6) and α-actinin-4, and more remain to be found. It is now possible to identify genetic causes underlying a proportion of nephrotic syndromes presenting at any age. The next big challenge for clinicians and researchers is to translate the molecular information learnt into the understanding of acquired, non-inherited forms of the disease and to guide therapeutic options. In this regard several exciting advances have been made, both in understanding the molecular mechanisms of current therapies and in revealing circulating plasma factors and the molecular pathways they trigger in the podocyte, that could be targeted by novel therapies.

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Titel: New developments in steroid-resistant nephrotic syndrome
verfasst von: Moin A. Saleem
Publikationsdatum: 01.05.2013
Verlag: Springer-Verlag
Erschienen in: Pediatric Nephrology / Ausgabe 5/2013
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-012-2239-0

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