title: Recent advances in Wilms tumor genetics.
abstract: The past decade has witnessed substantial growth in our knowledge of the genes and loci that are altered in Wilms tumor. Although Wilms tumor was one of the original paradigms of Knudson's two-hit model of cancer formation, it has become apparent that several genetic events contribute to Wilms tumorigenesis. Recent research has identified targets and regulators of the first Wilms tumor gene, WT1, has uncovered several candidate genes at the second Wilms tumor locus, WT2, and has identified two familial Wilms tumor loci, FWT1 and FWT2. The recent discovery of activating beta-catenin mutations in some Wilms tumors has also implicated the Wnt signaling pathway in this neoplasm. Recurrent abnormalities of other loci, including 16q, 1p, and 7p, have indicated that these sites may harbor Wilms tumor genes. An enhanced understanding of these and other genetic lesions will provide the foundation for novel targeted Wilms tumor therapies.
authors.affiliation: Department of Hematology and Oncology, St. Jude Children's Research Hospital, Tennessee 38105-2794, USA. jeff.dome@stjude.org
authors.forename: Jeffrey S
authors.initials: JS
authors.lastname: Dome
authors.forename: Max J
authors.initials: MJ
authors.lastname: Coppes
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000235:genetics; D002648:Child; D002878:Chromosomes, Human, Pair 1; D002885:Chromosomes, Human, Pair 16; D002897:Chromosomes, Human, Pair 7; D003598:Cytoskeletal Proteins; D016162:Genes, Wilms Tumor; D016158:Genes, p53; D006801:Humans; D007680:Kidney Neoplasms / Q000235:genetics*; D015534:Trans-Activators; D017624:WAGR Syndrome / Q000235:genetics; D009396:Wilms Tumor / Q000235:genetics*; D051176:beta Catenin
chemical_list: C495270:CTNNB1 protein, human; D003598:Cytoskeletal Proteins; D015534:Trans-Activators; D051176:beta Catenin