An X chromosome gene, WTX, is commonly inactivated in Wilms tumor.
Rivera MN,
Kim WJ,
Wells J,
Driscoll DR,
Brannigan BW,
Han M,
Kim JC,
Feinberg AP,
Gerald WL,
Vargas SO,
Chin L,
Iafrate AJ,
Bell DW,
Haber DA.
Massachusetts General Hospital Cancer Center, Harvard Medical Center, Boston, MA 02114, USA.
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.
PMID: 17204608 [PubMed - indexed for MEDLINE]