The candidate Wilms' tumour gene is involved in genitourinary development

@article{PritchardJones1990TheCW,
  title={The candidate Wilms' tumour gene is involved in genitourinary development},
  author={K. Pritchard-Jones and S. Fleming and D. Davidson and W. Bickmore and D. Porteous and C. Gosden and J. Bard and A. Buckler and J. Pelletier and D. Housman and V. Heyningen and N. 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… Expand
Multiple roles for the Wilms’ tumour suppressor gene, WT1 in genitourinary development
TLDR
It is now clear that mutations in this gene in humans can lead to abnormalities of the kidneys and gonads, as well as to the eponymous tumour, as revealed in knockout mice. Expand
The molecular biology of Wilms tumour.
TLDR
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. Expand
Investigation of the Wilms' tumour suppressor protein (WT1)
TLDR
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). Expand
The role of Wilms' tumor genes.
  • M. Hirose
  • Biology, Medicine
  • The journal of medical investigation : JMI
  • 1999
TLDR
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. Expand
Evolutionary comparison in the vertebrate lineage of WT1, a Wilms' tumour predisposition gene
TLDR
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. Expand
WT1 mutations contribute to abnormal genital system development and hereditary Wilms' tumour
TLDR
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. Expand
Wilms’ tumour - a case of disrupted development
TLDR
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. Expand
Expression of the Wilms' tumor suppressor gene WT1 during mouse embryogenesis.
TLDR
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. Expand
Wilms tumor and the WT1 gene.
TLDR
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. Expand
THE EXPRESSION PATTERN OF WILMS' TUMOUR GENE (WT1) PRODUCT IN NORMAL TISSUES AND PAEDIATRIC RENAL TUMOURS
TLDR
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. Expand
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References

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Familial predisposition to Wilms' tumour does not map to the short arm of chromosome 11
TLDR
A multipoint linkage analysis of a family segregating for Wilms' tumour, using polymorphic DNA markers mapped to chromosome lip implies an aetiological heterogeneity for Wiltshire tumour and raises questions concerning the general applicability of the carcinogenesis model. Expand
The β-subunit of follicle-stimulating hormone is deleted in patients with aniridia and Wilms' tumour, allowing a further definition of the WAGR locus
TLDR
Close physical linkage between FSHB and the WAGR locus is demonstrated, suggest a gene order for the four deleted markers and exclude other markers tested from this region, as well as testing genomic DNA from the hybrids with chromosome 11-specific probes. Expand
Homozygous deletion in Wilms tumours of a zinc-finger gene identified by chromosome jumping
TLDR
YTOGENETIC analysis has identified chromosome Ilpl3 as the smallest overlap region for deletions found in individuals with WAGR syndrome, which includes Wilms tumour, anirida, genito-urinary abnormalities and mental retardation. Expand
Lack of linkage of familial Wilms' tumour to chromosomal band 11 p13
TLDR
Investigating whether familial predisposition to WT is due to a germinal 11p13 mutation, which is distinct from the gene involved in tumorigensis and in WT predisposition in WT/aniridia 11p 13-deletion patients, found that familialWT predisposition was not genetically linked to any of the 11 p13 markers. Expand
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TLDR
Analysis of DNA from the cell lines and hybrids and with a cloned cDNA probe has shown that the catalase gene is deleted in four of five patients, and it is concluded these genes are likely to be outside the region 11p12-11p15. Expand
Familial Wiedemann-Beckwith syndrome and a second Wilms tumor locus both map to 11p15.5.
TLDR
Evidence is provided that familial WBS likely results from a defect at the same genetic locus as does its sporadic counterpart, and there is another locus, distinct from that involved in the WAGR syndrome, which plays a role in the association of Wilms tumor with WBS. Expand
Long range physical map of the Wilms' tumor-aniridia region on human chromosome 11
TLDR
A physical map of the relationship between genetic alterations at chromosomal band 11p13 and the WAGR (Wilms' tumor, aniridia, genitourinary anomalies, and mental retardation) syndrome is constructed using pulsed field gel electrophoresis. Expand
Two anonymous DNA segments distinguish the Wilms' tumor and aniridia loci.
TLDR
Two new anonymous DNA segments have been identified that map to the WAGR region of 11p13 that identify a cytologically undetectable deletion associated with a balanced chromosome translocation inherited by a patient with familial aniridia, but not Wilms' tumor. Expand
Chromosomal imbalance in the Aniridia-Wilms' tumor association: 11p interstitial deletion.
TLDR
The genetic heterogeneity of aniridia, the AGR triad, and Wilms' tumor are demonstrated, and Williams' tumor is indicated to be a neoplastic birth defect which can result from a variety of embryologic insults, some of which may be chromosomal or heritable. Expand
Infrequent genomic rearrangement and normal expression of the putative RB1 gene in retinoblastoma tumors.
TLDR
To extend the characterization of this gene, probes were used to search for genomic rearrang of DNA and to study the expression of the 4.7R gene in RB tumors, osteosarcoma tumors arising in RB patients, and other normal and malignant tissues. Expand
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