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WT1-mediated growth suppression of Wilms tumor cells expressing a WT1 splicing variant

D A Haber1, S Park, S Maheswaran

  • 1Laboratory of Molecular Genetics, Massachusetts General Hospital Cancer Center, Boston 02129.

Science (New York, N.Y.)
|December 24, 1993
PubMed

Insights

Researchers tested the tumor suppressor WT1 (Wilms tumor 1) in a human Wilms tumor cell line. They found a specific WT1 gene alteration in tumor cells, suggesting a new mechanism for Wilms tumor development.

Area of Science:

  • Molecular Biology
  • Genetics
  • Oncology

Background:

  • Wilms tumor is a pediatric kidney cancer.
  • WT1 (Wilms tumor 1) is a gene crucial for kidney development.
  • Mutations in WT1 are linked to Wilms tumor development.

Purpose of the Study:

  • To investigate the tumor suppressor activity of WT1.
  • To analyze WT1 gene expression in Wilms tumor cells.

Main Methods:

  • Developed a human Wilms tumor cell line (RM1).
  • Transfected RM1 cells with wild-type WT1 isoforms.
  • Analyzed WT1 transcript splicing in tumor cells and normal kidney tissue.

Main Results:

  • WT1 isoforms suppressed RM1 cell growth.
  • A specific splicing alteration (lacking exon 2) was found in endogenous WT1 transcripts in RM1 cells.
  • This alteration was present in varying amounts in all tested Wilms tumors but not in normal kidney tissue.

Conclusions:

  • The identified WT1 splicing alteration produces a functionally altered protein.
  • This abnormal transcript may represent a novel mechanism for WT1 inactivation in Wilms tumors.

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