The genomic organization and expression of the WT1 gene.

@article{Gessler1992TheGO,
  title={The genomic organization and expression of the WT1 gene.},
  author={Manfred Gessler and Arne K{\"o}nig and Gail A. P. Bruns},
  journal={Genomics},
  year={1992},
  volume={12 4},
  pages={
          807-13
        }
}
The Wilms tumor gene WT1, a proposed tumor suppressor gene, has been identified based on its location within a homozygous deletion found in tumor tissue. The gene encodes a putative transcription factor containing a Cys/His zinc finger domain. The critical homozygous deletions, however, are rarely seen, suggesting that in many cases the gene may be inactivated by more subtle alterations. To facilitate the search for smaller deletions and point mutations we have established the genomic… Expand
Genomic Organization of the Human WT1 Gene
TLDR
The genomic structure of the human WT1 gene, one of the recessive oncogenes for Wilms’tumor at chromosome lip 13, is analyzed to help analyze DNA abnormalities sometimes found in the tumors, as well as loss of heterozygosity. Expand
Sequence of the WT1 upstream region including the Wit-1 gene.
TLDR
To facilitate mapping of the WT1 promoter region and 5' control elements the authors have determined the sequence upstream of theWT1 transcription unit, which includes the Wit-1 gene that is transcribed in the opposite direction. Expand
Insertional inactivation of the WT1 gene in tumour cells from a patient with WAGR syndrome
The WT1 gene was analysed using DNA from a Wilms' tumour derived from a patient with the WAGR syndrome using single strand conformation polymorphism analysis and polymerase chain reaction sequencing.Expand
The Wilms' tumor 1 gene: oncogene or tumor suppressor gene?
TLDR
The role of the wt1 gene during normal development and homeostasis of several tissues and the involvement of the gene products in processes like apoptosis and tumorigenesis are discussed. Expand
Wilms tumor genes.
TLDR
The identification of several genes that are involved in the etiology or progression of WT, the preferential loss of maternally derived alleles in tumor tissue, and the observed reduction to 11p homozygosity in normal tissue DNA from some patients, all strikingly indicate that a simple, one-locus-'two-hit' genetic model for WT is inadequate. Expand
WT1 proteins: functions in growth and differentiation.
TLDR
The molecular mechanisms by which the various WT1 isoforms exert their functions in normal development are discussed and how alterations in WT1 may lead to developmental abnormalities and tumor growth are discussed. Expand
The Wilms' tumor suppressor WT1: approaches to gene function.
TLDR
The results suggest that ubiquitous expression of WT1 is not compatible with embryonic development and new approaches to understand WT1 function in vivo are discussed. Expand
Infrequent mutation of the WT1 gene in 77 Wilms' tumors
TLDR
The results suggest that direct alterations of the WTl gene are present in only a small fraction of Wilms' tumors, and mutations at other Wilm's tumor loci or disturbance of interactions between these genes likely play an important role inWilms' tumor development. Expand
Molecular mechanisms possibly affecting WT1 function in human ovarian tumors
TLDR
Findings invalidate the hypothesis that the WTI gene functions as a classical tumor‐suppressor gene in ovarian tumorigenesis and suggest that a different recessive oncogene may be “exposed” by the observed 11 p 13 allelic deletions. Expand
Wilms tumor genetics.
  • V. Huff
  • Biology, Medicine
  • American journal of medical genetics
  • 1998
TLDR
Determining the pattern of presence or absence of these various genetic alterations in tumors and elucidating the function of the genes involved will provide a better understanding of the cellular processes that are critical for normal cell growth and differentiation, but are abrogated in the course of tumorigenesis. Expand
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References

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An internal deletion within an 11p13 zinc finger gene contributes to the development of Wilms' tumor
TLDR
It is concluded that inactivation of this gene, which is primarily expressed in fetal kidney, is part of a series of events leading to the development of Wilms' tumor. Expand
Smallest region of overlap in Wilms tumor deletions uniquely implicates an 11p13 zinc finger gene as the disease locus.
TLDR
A partial deletion of the candidate gene is reported which, upon comparison with other documented homozygous deletions, permitted a precise definition of the critical genomic target in Wilms tumor. Expand
Evidence for WT1 as a Wilms tumor (WT) gene: intragenic germinal deletion in bilateral WT.
TLDR
DNA from both normal and tumor tissue from WT patients is analyzed and a patient with bilateral WT who is heterozygous for a small germinal deletion within this candidate gene is reported, supporting the identification of this locus as an 11p13 WT gene (WT1) and providing direct molecular data supporting the two-hit mutational model for WT. Expand
Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locus
TLDR
The genetic localization of this gene, its tissue-specific expression, and the function predicted from its sequence lead us to suggest that it represents the 11p13 Wilms' tumor gene. Expand
Structural rearrangements of the WT1 gene in Wilms' tumour cells.
TLDR
Analysis of cDNA from the candidate Wilms' predisposition gene, WT1, shows a partial homozygous deletion involving only the 3'-most exon of the gene, and three other tumours showed abnormally sized bands on Southern blot analysis which appear to reflect internal heterozygous rearrangements involving the 5' end of the genes. Expand
Role for the Wilms tumor gene in genital development?
TLDR
The molecular detection of a cytogenetically invisible 1-megabase deletion in an individual with aniridia, cryptorchidism, and hypospadias but no Wilms tumor but no WT implies that the WT gene exerts pleiotropic effect on both kidney and genitourinary development. Expand
Isolation, characterization, and expression of the murine Wilms' tumor gene (WT1) during kidney development.
TLDR
The narrow window of time during which WT1 is expressed at high levels in the kidney is consistent with the origin of Wilms' tumor from primitive nephroblasts and the postulated role of this gene as a negative regulator of growth. Expand
A tumor chromosome rearrangement further defines the 11p13 Wilms tumor locus.
TLDR
Findings strengthen previous conclusions regarding the obligate location for the 11p13 WT locus and support the suggestion that the Wilms tumor gene has been cloned. 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
Binding of the Wilms' tumor locus zinc finger protein to the EGR-1 consensus sequence.
TLDR
Results suggest that the WTL protein may act at the DNA binding site of a growth factor-inducible gene and that loss of DNA-binding activity contributes to the tumorigenic process. Expand
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