Article
Murine Denys-Drash syndrome: evidence of podocyte de-differentiation and systemic mediation of glomerulosclerosis.
Human molecular genetics - 15 Sept 2003
Patek Charles E, Fleming Stewart, Miles Colin G, Bellamy Christopher O, Ladomery Michael, Spraggon Lee, Mullins John, Hastie Nicholas D, Hooper Martin L
Abstract excerpt
Denys-Drash syndrome (DDS) is caused by dominant mutations of the Wilms' tumour suppressor gene, WT1, and characterized by a nephropathy involving diffuse mesangial sclerosis, male pseudohermaphroditism and/or Wilms' tumourigenesis. Previously, we reported that heterozygosity for the Wt1tmT396 mutation induces DDS in heterozygous and chimeric (Wt1tmT396/+<-->+/+) mice. In the present study, the fate of Wt1 mutant...
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