Wilms' tumour: reconciling genetics and biology

Trends Genet. 1992 Jan;8(1):16-21. doi: 10.1016/0168-9525(92)90019-z.

Abstract

Wilms' tumour, a paediatric malignancy of the kidney, is a striking example of the relationship between aberrant development and cancer. Several different genetic loci have been implicated in the aetiology of the tumour; genomic imprinting also plays a role. One Wilms' tumour predisposition gene (WT1), encoding a zinc finger protein, is expressed in a limited set of tissues, including developing nephrons and gonads. The biology and genetics of Wilms' tumour underline the developmental relationship between kidneys and gonads.

Publication types

  • Review

MeSH terms

  • Abnormalities, Multiple / genetics
  • Aniridia / genetics
  • Chromosomes, Human, Pair 11
  • Cryptorchidism / genetics
  • DNA-Binding Proteins / physiology
  • Female
  • Gene Expression Regulation, Developmental*
  • Gene Expression Regulation, Neoplastic*
  • Genes, Wilms Tumor*
  • Gonads / embryology
  • Humans
  • Hypospadias / genetics
  • Kidney / embryology*
  • Kidney Neoplasms / embryology
  • Kidney Neoplasms / genetics*
  • Male
  • Oncogenes
  • Sequence Deletion
  • Syndrome
  • Transcription Factors / physiology
  • WT1 Proteins
  • Wilms Tumor / embryology
  • Wilms Tumor / genetics*
  • Zinc Fingers

Substances

  • DNA-Binding Proteins
  • Transcription Factors
  • WT1 Proteins