Article
Homozygous inactivation of WT1 in a Wilms' tumor associated with the WAGR syndrome.
Genes, chromosomes & cancer - 1 Jul 1993
Gessler M, König A, Moore J, Qualman S, Arden K, Cavenee W, Bruns G
Abstract excerpt
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...
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