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

doi:10.1126/science.1137509

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

School of Medicine

Research output: Contribution to journal › Article › peer-review

268 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 language	English (US)
Pages (from-to)	642-645
Number of pages	4
Journal	Science
Volume	315
Issue number	5812
DOIs	https://doi.org/10.1126/science.1137509
State	Published - Feb 2 2007

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@article{25e0a19950d34c82b6948f51116daafa,

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

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.",

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

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language = "English (US)",

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T1 - An X chromosome gene, WTX, is commonly inactivated in wilms tumor

AU - Rivera, Miguel N.

AU - Woo, Jae Kim

AU - Wells, Julie

AU - Driscoll, David R.

AU - Brannigan, Brian W.

AU - Han, Moonjoo

AU - Kim, James C.

AU - Feinberg, Andrew P.

AU - Gerald, William L.

AU - Vargas, Sara O.

AU - Chin, Lynda

AU - Iafrate, A. John

AU - Bell, Daphne W.

AU - Haber, Daniel A.

PY - 2007/2/2

Y1 - 2007/2/2

N2 - 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.

AB - 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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SP - 642

EP - 645

JO - Science

JF - Science

IS - 5812

ER -

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

Abstract

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