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© 1994 Oxford University Press

OTHER

The Wilms tumour (WT1) gene is mutated in a secondary leukaemia in a WAGR patient

K. Pritchard-Jones*, J. Renshaw and L. King-Underwood

Section of Paediatrics, Institute of Cancer Research/Royal Marsden NHS Trust Downs Road, Sutton, Surrey SM2 5PT, UK

*To whom correspondence should be addressed

Received May 23, 1994; Revised July 1, 1994; Accepted July 1, 1994

The Wilms tumour (WT1) gene was first localized through its deletion in individuals with the WAGR syndrome (Wilms tumour, aniridia, genitourinary abnormalities and mental retardation). Such individuals have a 30–50% lifetime risk of developing Wilms tumour and carry constitutional interstitial deletions of chromosome 11p13, including the WT1 gene. Second primary tumours occurring in such individuals might also be related to their genetic predisposition to cancer, as shown for hereditary retinoblastoma. We have found a mutation in the zinc finger region of the remaining WT1 allele in a case of acute myeloid leukaemia developing in a Wilms tumour survivor with the WAGR syndrome. This mutation would be predicted to disrupt DNA binding by this developmentally regulated transcription factor. This finding implicates the WT1 gene in the regulation of myelopoiesis and suggests that WT1 mutations may be found in some sporadic leukaemias.


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