Proceedings of the National Academy of Sciences · 1996 · 218 citations · 41 references
Life SciencesGeneticsMolecular BiologyCancer GeneticsGene ExpressionTranscriptional RegulationNoncoding Rna In CancerUntranslated RegionsWilms TumorZinc Finger NucleasesDenys-drash SyndromeDna Binding SitesRna-binding MotifSubnuclear LocalizationWilms Tumor 1Zinc Binding ProteinsZinc Finger MotifsZinc Finger Proteins
The Wilms tumor suppressor gene WT1 is implicated in the ontogeny of genito-urinary abnormalities, including Denys-Drash syndrome and Wilms tumor of the kidney. WT1 encodes Kruppel-type zinc finger proteins that can regulate the expression of several growth-related genes, apparently by binding to specific DNA sites located within 5' untranslated leader regions as well as 5' promoter sequences. Both WT1 and a closely related early growth response factor, EGR1, can bind the same DNA sequences from the mouse gene encoding insulin-like growth factor 2 (Igf-2). We report that WT1, but not EGR1, can bind specific Igf-2 exonic RNA sequences, and that the zinc fingers are required for this interaction. WT1 zinc finger 1, which is not represented in EGR1, plays a more significant role in RNA binding than zinc finger 4, which does have a counterpart in EGR1. Furthermore, the normal subnuclear localization of WT1 proteins is shown to be RNase, but not DNase, sensitive. Therefore, WT1 might, like the Kruppel-type zinc finger protein TFIIIA, regulate gene expression by both transcriptional and posttranscriptional mechanisms.
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WT-1 is required for early kidney development
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