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. 2007 Feb 2;315(5812):642-5.
doi: 10.1126/science.1137509. Epub 2007 Jan 4.

An X chromosome gene, WTX, is commonly inactivated in Wilms tumor

Miguel N Rivera  1 , Woo Jae Kim, Julie Wells, David R Driscoll, Brian W Brannigan, Moonjoo Han, James C Kim, Andrew P Feinberg, William L Gerald, Sara O Vargas, Lynda Chin, A John Iafrate, Daphne W Bell, Daniel A Haber
Affiliations

An X chromosome gene, WTX, is commonly inactivated in Wilms tumor

Miguel N Rivera et al. Science. .

Abstract

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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