Article
A mutant form of the Wilms' tumor suppressor gene WT1 observed in Denys-Drash syndrome interferes with glomerular capillary development.
Journal of the American Society of Nephrology : JASN - 1 Aug 2002
Natoli Thomas A, Liu Jing, Eremina Vera, Hodgens Karen, Li Cong, Hamano Yuki, Mundel Peter, Kalluri Raghu, Miner Jeffrey H, Quaggin Susan E, Kreidberg Jordan A
Abstract excerpt
The Wilms' tumor suppressor gene WT1 encodes a zinc finger protein that is required for urogenital development. In the kidney, WT1 is most highly expressed in glomerular epithelial cells or podocytes, which are an essential component of the filtering system. Human subjects heterozygous for point mutations in the WT1 gene develop renal failure because of the formation of scar tissue within glomeruli. The...
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