Article
Impaired glomerular maturation and lack of VEGF165b in Denys-Drash syndrome.
Journal of the American Society of Nephrology : JASN - 1 Mar 2007
Schumacher Valérie Anne, Jeruschke Stefanie, Eitner Frank, Becker Jan Ulrich, Pitschke Gerald, Ince Yasemin, Miner Jeffrey H, Leuschner Ivo, Engers Rainer, Everding Anne Schulze, Bulla Monika, Royer-Pokora Brigitte
Abstract excerpt
Individuals with Denys-Drash syndrome (DDS) develop diffuse mesangial sclerosis, ultimately leading to renal failure. The disease is caused by mutations that affect the zinc finger structure of the Wilms' tumor protein (WT1), but the mechanisms whereby these mutations result in glomerulosclerosis remain largely obscure. How WT1 regulates genes is likely to be complex, because it has multiple splice forms, binds...
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