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The FASEB Journal, Vol 7, 896-903, Copyright © 1993 by The Federation of American Societies for Experimental Biology

REVIEWS

The WT1 Wilms tumor gene product: a developmentally regulated transcription factor in the kidney that functions as a tumor suppressor

FJ Rauscher 3d
Wistar Institute of Anatomy and Biology, Philadelphia, Pennsylvania 19104.

Alteration of transcription factor function is becoming a common theme in molecular mechanisms of oncogenesis. A recent example of this trend is the isolation and characterization of the chromosome 11p13 Wilms tumor suppressor gene, WT1. The WT1 protein contains a DNA binding domain consisting of four zinc fingers of the Cys2-His2 class and a proline-glutamine rich region capable of regulating transcription. Deletions of the WT1 gene or point mutations which destroy the DNA binding activity of the protein are associated with the development of the pediatric nephroblastoma Wilms tumor and Denys-Drash syndrome. This article reviews the role of WT1 in normal kidney development processes, the known biochemical functions of the protein and the status of identifying target genes regulated by this potentially oncogenic transcription factor.


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[Abstract] [Full Text] [PDF]

Home page
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Home page
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[Abstract] [Full Text] [PDF]

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Home page
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J. Cell Sci., May 1, 2003; 116(9): 1653 - 1658.
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Home page
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Home page
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Home page
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Home page
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Home page
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Home page
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Am. J. Pathol., March 1, 1999; 154(3): 635 - 647.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
E. S. Silverman and T. Collins
Pathways of Egr-1-Mediated Gene Transcription in Vascular Biology
Am. J. Pathol., March 1, 1999; 154(3): 665 - 670.
[Full Text] [PDF]

Home page
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[Abstract] [Full Text] [PDF]

Home page
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Home page
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[Abstract] [Full Text] [PDF]

Home page
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J. Biol. Chem., September 25, 1998; 273(39): 25472 - 25479.
[Abstract] [Full Text] [PDF]

Home page
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J. Biol. Chem., May 1, 1998; 273(18): 10880 - 10887.
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Home page
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Inhibition of cellular proliferation by the Wilms tumor suppressor WT1 requires association with the inducible chaperone Hsp70
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Home page
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Altered expression of the WT1 Wilms tumor suppressor gene in human breast cancer
PNAS, July 22, 1997; 94(15): 8132 - 8137.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
G. Dong, R. Rajah, T. Vu, A. R. Hoffman, R. G. Rosenfeld, C. T. Roberts Jr., D. M. Peehl, and P. Cohen
Decreased Expression of Wilms' Tumor Gene WT-1 and Elevated Expression of Insulin Growth Factor-II (IGF-II) and Type 1 IGF Receptor Genes in Prostatic Stromal Cells from Patients with Benign Prostatic Hyperplasia
J. Clin. Endocrinol. Metab., July 1, 1997; 82(7): 2198 - 2203.
[Abstract] [Full Text] [PDF]

Home page
 EndocrinologyHome page
S. R. Plymate, V. L. Bae, L. Maddison, L. S. Quinn, and J. L. Ware
Reexpression of the Type 1 Insulin-Like Growth Factor Receptor Inhibits the Malignant Phenotype of Simian Virus 40 T Antigen Immortalized Human Prostate Epithelial Cells
Endocrinology, April 1, 1997; 138(4): 1728 - 1735.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
E. Karnieli, H. Werner, F. J. Rauscher III, L. E. Benjamin, and D. LeRoith
The IGF-I Receptor Gene Promoter Is a Molecular Target for the Ewing's Sarcoma-Wilms' Tumor 1Fusion Protein
J. Biol. Chem., August 9, 1996; 271(32): 19304 - 19309.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
W. Bruening and J. Pelletier
A Non-AUG Translational Initiation Event Generates Novel WT1 Isoforms
J. Biol. Chem., April 12, 1996; 271(15): 8646 - 8654.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
A. J. Williams, L. M. Khachigian, T. Shows, and T. Collins
Isolation and Characterization of a Novel Zinc-finger Protein with Transcriptional Repressor Activity
J. Biol. Chem., September 22, 1995; 270(38): 22143 - 22152.
[Abstract] [Full Text] [PDF]

Home page
 NEJMHome page
M. J. Coppes, D. A. Haber, and P. E. Grundy
Genetic Events in the Development of Wilms' Tumor
N. Engl. J. Med., September 1, 1994; 331(9): 586 - 590.
[Full Text]

Home page
 J. Biol. Chem.Home page
W. Wang, S. B. Lee, R. Palmer, L. W. Ellisen, and D. A. Haber
A Functional Interaction with CBP Contributes to Transcriptional Activation by the Wilms Tumor Suppressor WT1
J. Biol. Chem., May 11, 2001; 276(20): 16810 - 16816.
[Abstract] [Full Text] [PDF]


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Copyright © 1993 by The Federation of American Societies for Experimental Biology.