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Rivera, Miguel N; Kim, Woo Jae; Wells, Julie; Driscoll, David R; Brannigan, Brian W; Han, Moonjoo; Kim, James C; Feinberg, Andrew P; Gerald, William L; Vargas, Sara O; Chin, Lynda; Iafrate, A. John; Bell, Daphne W; Haber, Daniel A
Science (American Association for the Advancement of Science), 02/2007, Letnik: 315, Številka: 5812Journal Article
Wilms tumor is a pediatric kidney cancer associated with inactivation of the WT1 tumor-suppressor gene in 5 to 10% of cases. Using a high-resolution screen for DNA copy-number alterations in Wilms tumor, we identified somatic deletions targeting a previously uncharacterized gene on the X chromosome. This gene, which we call WTX, is inactivated in approximately one-third of Wilms tumors (15 of 51 tumors). Tumors with mutations in WTX lack WT1 mutations, and both genes share a restricted temporal and spatial expression pattern in normal renal precursors. In contrast to biallelic inactivation of autosomal tumor-suppressor genes, WTX is inactivated by a monoallelic "single-hit" event targeting the single X chromosome in tumors from males and the active X chromosome in tumors from females.
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