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Grant Details

Grant Number: 5R01CA078257-05 Interpret this number
Primary Investigator: Huff, Vicki
Organization: Ut Md Anderson Cancer Ctr
Project Title: Molecular Investigation of Familial Wilms Tumor
Fiscal Year: 2003
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DESCRIPTION: (Adapted from investigator's abstract) A revised application to test the hypothesis that tumorigenesis in Wilms tumor results from mutations, either germline or somatic, at more than one locus in the tumor cell. One Wilms tumor gene is known: the transcription factor WT1 on chromosome 11p. The PI proposes to fine map a second locus, WT2, for hereditary Wilms tumor on chr19q and identify the responsible gene. Specific Aims are: (1) Ascertain and sample additional families with familial WT; (2) identify the subset that show linkage to 19q (as well as any families linked to other regions implicated in WT); (3) explore all familial cases, whether linked to 19q or not, for LOH involving the critical region on 19q or other regions implicated in WT; (4) refine the position of the 19q WT2 locus on a physical map of the region using meiotic recombination, loss of heterozygosity, and disequilibrium approaches; and (5) screen candidate genes and ESTs in the region for germline and somatic mutations in WT patients

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The Wilms tumor gene, Wt1, is required for Sox9 expression and maintenance of tubular architecture in the developing testis.
Authors: Gao F. , Maiti S. , Alam N. , Zhang Z. , Deng J.M. , Behringer R.R. , Lécureuil C. , Guillou F. , Huff V. .
Source: Proceedings Of The National Academy Of Sciences Of The United States Of America, 2006-08-08 00:00:00.0; 103(32), p. 11987-92.
EPub date: 2006-08-08 00:00:00.0.
PMID: 16877546
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Genome-wide loss of heterozygosity analysis of WT1-wild-type and WT1-mutant Wilms tumors.
Authors: Ruteshouser E.C. , Hendrickson B.W. , Colella S. , Krahe R. , Pinto L. , Huff V. .
Source: Genes, Chromosomes & Cancer, 2005 Jun; 43(2), p. 172-80.
PMID: 15761866
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The Wt1+/R394W mouse displays glomerulosclerosis and early-onset renal failure characteristic of human Denys-Drash syndrome.
Authors: Gao F. , Maiti S. , Sun G. , Ordonez N.G. , Udtha M. , Deng J.M. , Behringer R.R. , Huff V. .
Source: Molecular And Cellular Biology, 2004 Nov; 24(22), p. 9899-910.
PMID: 15509792
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Twenty-four new cases of WT1 germline mutations and review of the literature: genotype/phenotype correlations for Wilms tumor development.
Authors: Royer-Pokora B. , Beier M. , Henzler M. , Alam R. , Schumacher V. , Weirich A. , Huff V. .
Source: American Journal Of Medical Genetics. Part A, 2004-06-15 00:00:00.0; 127A(3), p. 249-57.
PMID: 15150775
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Upregulation of c-MYC in WT1-mutant tumors: assessment of WT1 putative transcriptional targets using cDNA microarray expression profiling of genetically defined Wilms' tumors.
Authors: Udtha M. , Lee S.J. , Alam R. , Coombes K. , Huff V. .
Source: Oncogene, 2003-06-12 00:00:00.0; 22(24), p. 3821-6.
PMID: 12802290
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Myc Target In Myeloid Cells-1, A Novel C-myc Target, Recapitulates Multiple C-myc Phenotypes
Authors: Yin X. , Grove L. , Rogulski K. , Prochownik E.V. .
Source: The Journal Of Biological Chemistry, 2002-05-31 00:00:00.0; 277(22), p. 19998-20010.
PMID: 11909865
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Absence of PPP2R1A mutations in Wilms tumor.
Authors: Ruteshouser E.C. , Ashworth L.K. , Huff V. .
Source: Oncogene, 2001-04-12 00:00:00.0; 20(16), p. 2050-4.
PMID: 11360189
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Pulmonary Dysplasia, Denys-drash Syndrome And Wilms Tumor 1 Gene Mutation In Twins
Authors: Dharnidharka V.R. , Ruteshouser E.C. , Rosen S. , Kozakewich H. , Harris H.W. , Herrin J.T. , Huff V. .
Source: Pediatric Nephrology (berlin, Germany), 2001 Mar; 16(3), p. 227-31.
PMID: 11322369
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Frequent association of beta-catenin and WT1 mutations in Wilms tumors.
Authors: Maiti S. , Alam R. , Amos C.I. , Huff V. .
Source: Cancer Research, 2000-11-15 00:00:00.0; 60(22), p. 6288-92.
PMID: 11103785
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