Abstract
WILMS' tumour is an embryonic kidney tumour thought to arise through aberrant mesenchymal stem cell differentiation1 and to result from loss of function of a 'tumour suppressor' gene(s)2. Both sporadic and syndrome-associated Wilms' tumours are accompanied by an increased frequency of abnormalities of the urinary tract and genitalia3. Deletional analysis of individuals with the WAGR syndrome4–8 (for, Wilms' tumour, aniridia, genitourinary abnormalities and mental retardation) showed that a Wilms' tumour gene lies at chromosomal position 11p13. This led to the isolation of a candidate Wilms' tumour gene9,10, encoding a zinc-finger protein which is likely to be a transcription factor. To gain insight into the role of this candidate gene in normal development and tumorigenesis, we have now performed in situ messenger RNA hybridization on sections of human embryos and Wilms' tumours. The candidate Wilms' tumour gene is expressed specifically in the condensed mesenchyme, renal vesicle and glomerular epithelium of the developing kidney, in the related mesonephric glomeruli and in cells approximating these structures in tumours. The other main sites of expression are the genital ridge, fetal gonad and mesothelium. These data suggest that (1) this candidate is indeed a Wilms' tumour gene, (2) the associated genital abnormalities are pleiotropic effects of mutation in the Wilms' tumour gene itself, in support of recent genetic analysis11, and (3) this gene has a specific role in kidney development and a wider role in mesenchymal–epithelial transitions.
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Pritchard-Jones, K., Fleming, S., Davidson, D. et al. The candidate Wilms' tumour gene is involved in genitourinary development. Nature 346, 194–197 (1990). https://doi.org/10.1038/346194a0
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DOI: https://doi.org/10.1038/346194a0
