Other Loci Implicated in Wilms Tumor

  • Max J. Coppes
  • Christine Campbell
  • Bryan R. G. Williams
Part of the Molecular Biology Intelligence Unit book series (MBIU)


There are several features of Wilms tumor and WT1 mutations that make it clear that the original two-hit hypothesis proposed by Knudson and Strong1 is inadequate to explain the etiology of this tumor. Although inactivation as proposed in the two-hit hypothesis of the WT1 gene has indeed been described in certain Wilms tumors (see chapter 7), its incidence (less than 15%) indicates the likely existence of alternative loci involved in the development of this pediatric renal malignancy. Moreover, at least some Wilms tumors are heterozygous for the mutant WT1 allele: that is, tumor initiation did not require the loss of both wild-type alleles (see chapter 7).


Antisense Transcript IGF2 Gene Natl Cancer Lnst WAGR Syndrome Nephrogenic Rest 
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Copyright information

© Springer-Verlag Berlin Heidelberg 1995

Authors and Affiliations

  • Max J. Coppes
    • 1
  • Christine Campbell
    • 2
  • Bryan R. G. Williams
    • 2
  1. 1.Tom Baker Cancer Centre and University of CalgaryCalgaryCanada
  2. 2.Department of Cancer Biology Research InstituteCleveland Clinic FoundationClevelandUSA

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