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Keywords:

  • cancer genetics;
  • Denys–Drash syndrome;
  • gene targeting;
  • retinoblastoma;
  • tumour suppressor gene

Tumour suppressor genes prevent cancer development. They can be identified by studying humans, but a full understanding of the mechanisms of their action requires the production of animal models. Mice with mutations in tumour suppressor genes can be produced by gene targeting. The phenotypic consequences of tumour suppressor gene mutations in mice and humans show parallels and contrasts, and both can contribute to the elucidation of disease processes.