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Science 2 February 2007:
Vol. 315. no. 5812, p. 569
DOI: 10.1126/science.315.5812.569h
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This Week in Science

Wilms tumor is a pediatric kidney cancer that can be inherited or arise sporadically. A small fraction of sporadic cases are caused by mutations in the WT1 gene on chromosome 11, which codes for a transcription factor regulating kidney development. Rivera et al. (p. 642, published online 4 January) now show that sporadic forms of Wilms tumor can also arise from mutations in a gene on the X chromosome, WTX. The function of the WTX protein is not yet known, but the gene's location on the X chromosome is of particular interest. Inactivation of most tumor suppressor genes requires two separate events or hits. Because humans carry only one functional allele of all X chromosome genes (in females one allele of each gene is silenced), the WTX gene presumably can be disabled by a single hit. The discovery of WTX suggests that X chromosome genes may play underappreciated roles in human cancer.




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Science. ISSN 0036-8075 (print), 1095-9203 (online)