WT1 is a DNA Binding Protein Containing Four C2H2 Zinc Fingers. 89 The DNA Binding Domain ofWT1 ··············································~··· 90 WT1 Inhibits Tag and SV40 Origin Dependent Replication. 97 WT1 is an Unusual C2H2 Zinc Finger Protein. 98 WT1 is a Transcriptional Regulator. 99 Possible Downstream Targets ofWT1 Transcriptional Regulation. 103 Protein-Protein Interactions Involving WT1. 104 Other Factors that May Contribute to or Modify the Cellular Function ofWT1. 104 WT1 and Apoptosis. 106 Conclusion. 107 7. Naturally Occurring Mutations in the WTJ Gene. 113 Introduction. 113 WTI Mutations in Wilms Tumor. 114 Analysis of WTI Mutations in Tumors Other than Wilms Tumor. 122 Constitutional WTI Mutations and the Denys-Drash Syndrome. 125 Conclusion. 131 8. Other Loci Implicated in Wtlms Tumor. 137 Introduction. 137 Nephrogenic Rests, WTI Mutations and Wilms Tumor. 137 Undetectable WTI Mutations. 138 Wilms Tumor and WITI. 140 Loss of Heterozygosity for Chromosome 1 p and 16q. 141 Wilms Tumor and Beckwith-Wiedemann Syndrome. 141 Wilms Tumor and Perlman Syndrome. 144 Imprinting, IGF/l, HI9 and Wilms Tumor. 144 Wilms Tumor and Li-F raumeni Syndrome. 149 Familial Wilms Tumor. 149 Wilms Tumor and Other Disease Associations. 150 Conclusion. 150 Index.

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