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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 Tumorand Other Disease Associations................................. 150 Conclusion........................................................................................ 150 Index.............................................................................