The candidate Wilms' tumour gene is involved in genitourinary development

@article{PritchardJones1990TheCW,
  title={The candidate Wilms' tumour gene is involved in genitourinary development},
  author={Kathryn Pritchard-Jones and Stewart Fleming and Duncan Davidson and Wendy A. Bickmore and David Porteous and Christine M. Gosden and Jonathan B. L. Bard and Alan Buckler and Jerry Pelletier and David E. Housman and Veronica van Heyningen and Nicholas D. Hastie},
  journal={Nature},
  year={1990},
  volume={346},
  pages={194-197}
}
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… 

The molecular biology of Wilms tumour.

WT serves as a paradigm for understanding the relationship between loss of developmental control and gain of tumourigenic potential, and some loci have been shown to be associated with particular clinical outcomes, suggesting that they might be used to determine prognosis.

Investigation of the Wilms' tumour suppressor protein (WT1)

The N-terminus of WT1 was investigated to determine the minimal region required for self association, and a functional assay was designed to determined the DNA binding fractional activity of the WT1 zinc finger domain variants used by Fagerlund (2009).

Evolutionary comparison in the vertebrate lineage of WT1, a Wilms' tumour predisposition gene

WTI analysis in Wilms' tumours revealed that both functional copies are lost in about 10% of sporadic Wilm's tumours, confirming the role of WTI as a tumour suppressor gene in at least some cases, following Knudson's two hit hypothesis for tumorigenesis.

The role of Wilms' tumor genes.

  • M. Hirose
  • Medicine, Biology
    The journal of medical investigation : JMI
  • 1999
A correlated expression between WT1 and mdr-1 in vincristine resistant cells indicates a close relation with multi-drug resistance and is a promising diagnostic marker for chemoresistance in hematologic malignancies.

WT1 mutations contribute to abnormal genital system development and hereditary Wilms' tumour

Constitutional mutations within the WT1 genes of two individuals with a combination of WT and genital abnormalities are reported as evidence of a role for a recessive oncogene in mammalian development.

Wilms’ tumour - a case of disrupted development

How one of the normal functions of WT1 may be to suppress myogenesis during kidney development is discussed and how YAC (yeast artificial chromosome) transgenesis to analyse WT1 regulation and function in mice is described.

Expression of the Wilms' tumor suppressor gene WT1 during mouse embryogenesis.

In situ mRNA hybridization and immunohistochemistry is used to examine WT1 expression in murine embryos during the period prior to and throughout active organogenesis to better define the temporal and spatial distribution ofWT1 expression during embryogenesis.

Wilms tumor and the WT1 gene.

Understanding the distinct functional properties of WT1 isoforms and tumor-associated variants will provide unique insight into the link between normal organ-specific differentiation and malignancy.

THE EXPRESSION PATTERN OF WILMS' TUMOUR GENE (WT1) PRODUCT IN NORMAL TISSUES AND PAEDIATRIC RENAL TUMOURS

A sensitive immunohistochemical technique was established to localize the WT1 gene product in archival normal tissues and paediatric renal tumour samples, providing an insight into the interrelationships of these renal tumours.
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References

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Homozygous deletion in Wilms tumours of a zinc-finger gene identified by chromosome jumping

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