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

Miguel N. Rivera, Jae Kim Woo, 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

Research output: Contribution to journalArticle

263 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)642-645
Number of pages4
JournalScience
Volume315
Issue number5812
DOIs
StatePublished - Feb 2 2007
Externally publishedYes

ASJC Scopus subject areas

  • General

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    Rivera, M. N., Woo, J. K., Wells, J., Driscoll, D. R., Brannigan, B. W., Han, M., Kim, J. C., Feinberg, A. P., Gerald, W. L., Vargas, S. O., Chin, L., Iafrate, A. J., Bell, D. W., & Haber, D. A. (2007). An X chromosome gene, WTX, is commonly inactivated in wilms tumor. Science, 315(5812), 642-645. https://doi.org/10.1126/science.1137509