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

Abstract
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.
AuthorsF J Rauscher 3rd
JournalFASEB journal : official publication of the Federation of American Societies for Experimental Biology (FASEB J) Vol. 7 Issue 10 Pg. 896-903 (Jul 1993) ISSN: 0892-6638 [Print] United States
PMID8393820 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S., Review)
Chemical References
  • DNA-Binding Proteins
  • Transcription Factors
  • WT1 Proteins
  • DNA
Topics
  • Amino Acid Sequence
  • Animals
  • Base Sequence
  • Chromosomes, Human, Pair 11
  • DNA (metabolism)
  • DNA-Binding Proteins (genetics, metabolism, physiology)
  • Genes, Wilms Tumor
  • Humans
  • Kidney (growth & development, metabolism)
  • Molecular Sequence Data
  • Transcription Factors (metabolism)
  • WT1 Proteins
  • Wilms Tumor (genetics)

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