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Papers In Press, published online ahead of print February 20, 2001
J. Biol. Chem, 10.1074/jbc.M009056200
Submitted on October 4, 2000
Revised on February 20, 2001
Accepted on February 17, 2001
Biochemistry and Molecular Biology, M.D. Anderson Cancer Center, Houston, TX 77030
Corresponding Author: gsaunders{at}odin.mdacc.tmc.edu
The product of the Wilms? tumor gene, WT1, is essential for male sex determination and differentiation in mammals. In addition to causing Wilms? tumor, mutations in WT1 often cause two distinct but overlapping urogenital defects in men Denys-Drash syndrome (DDS) and Frasier syndrome. In this study we investigated the regulation of the sex determination gene SRY by WT1. Our results showed that WT1 up-regulates the SRY gene through the proximal EGR-1-like DNA-binding sequences in the core promoter. Mutant WT1 proteins in DDS patients were unable to activate this promoter. These mutants did not act in a dominant negative manner, as expected over the wild type WT1 in this promoter. We also found that WT1 could transactivate the endogenous SRY gene. These observations, together with the overlapping expression patterns of WT1 and SRY in human gonads, led us to propose that WT1 regulates SRY in the initial sex-determination process in humans and activates a cascade of genes ultimately leading to the complete organogenesis of the testis.
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