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  • Title: Homozygous inactivation of WT1 in a Wilms' tumor associated with the WAGR syndrome.
    Author: Gessler M, König A, Moore J, Qualman S, Arden K, Cavenee W, Bruns G.
    Journal: Genes Chromosomes Cancer; 1993 Jul; 7(3):131-6. PubMed ID: 7687865.
    Abstract:
    Wilms' tumor is a childhood nephroblastoma that is postulated to arise through the inactivation of a tumor suppressor gene by a two-hit mechanism. A candidate 11p13 Wilms' tumor gene, WT1, has been cloned and shown to encode a zinc finger protein. Patients with the WAGR syndrome (Wilm's tumor, aniridia, genitourinary abnormalities, and mental retardation) have a high risk of developing Wilms' tumor and they carry constitutional deletions of one chromosome 11 allele encompassing the WT1 gene. Analysis of the remaining WT1 allele in a Wilms' tumor from a WAGR patient revealed the deletion of a single nucleotide in exon 7. This mutation likely played a key role in tumor formation, as it prevents translation of the DNA-binding zinc finger domain that is essential for the function of the WT1 polypeptide as a transcriptional regulator.
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