Novel Oncogenic Mutations in the WT1 Wilms' Tumor Suppressor Gene: A t(11;22) Fuses the Ewing's Sarcoma Gene, EWS1, to WT1 in Desmoplastic Small Round Cell Tumor
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Excerpt
The differentiated phenotype of a cell is largely determined by the set of genes that are actively being transcribed at a given moment. Hence, the set of nuclear transcription factors present and active that control expression of those target genes specify the output from this system and therefore the cellular phenotype. Since a single transcription factor may regulate hundreds of “downstream” target genes via binding to their regulatory promoter/enhancer elements, inactivation or alteration of DNA binding or transcriptional regulatory functions (via mutation or deletion) could result in catastrophic effects on cellular phenotype or proliferative potential. Thus, it is not surprising that alteration of transcription factor expression/function has emerged as a major theme among molecular mechanisms of oncogenesis (Lewin 1991). Many examples of sequence-specific DNA-binding proteins with oncogenic potential have been encountered in the guise of viral/cellular oncogenes, tumor suppressor genes, and developmental control genes (Bishop 1991). Many of these proto-oncogene...
