Report

An X Chromosome Gene, WTX, Is Commonly Inactivated in Wilms Tumor

See allHide authors and affiliations

Science  02 Feb 2007:
Vol. 315, Issue 5812, pp. 642-645
DOI: 10.1126/science.1137509

You are currently viewing the abstract.

View Full Text

Log in to view the full text

Log in through your institution

Log in through your institution

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.

    View Full Text