D
IPR000976

Wilm's tumour protein, N-terminal

InterPro entry
Short nameWilms_tumour_N

Description

Wilm's tumour (WT) is an embryonal malignancy of the kidney, affecting around 1 in 10,000 infants. It occurs in both sporadic and hereditary forms. Inactivation of WT1 is one of the causes of Wilm's tumour. Defects in the WT1 gene are also associated with Denys-Drash Syndrome (DDS), which is characterised by typical nephropathy and genital abnormalities. The WT1 gene product shows similarity to the zinc fingers of the mammalian growth regulated EGR1 and EGR2 proteins
[1, 2, 3, 4]
.

References

1.The WT1 Wilms tumor gene product: a developmentally regulated transcription factor in the kidney that functions as a tumor suppressor. Rauscher FJ 3rd. FASEB J. 7, 896-903, (1993). View articlePMID: 8393820

2.Isolation, characterization, and expression of the murine Wilms' tumor gene (WT1) during kidney development. Buckler AJ, Pelletier J, Haber DA, Glaser T, Housman DE. Mol. Cell. Biol. 11, 1707-12, (1991). View articlePMID: 1671709

3.Homozygous deletion in Wilms tumours of a zinc-finger gene identified by chromosome jumping. Gessler M, Poustka A, Cavenee W, Neve RL, Orkin SH, Bruns GA. Nature 343, 774-8, (1990). View articlePMID: 2154702

4.Zinc finger point mutations within the WT1 gene in Wilms tumor patients. Little MH, Prosser J, Condie A, Smith PJ, Van Heyningen V, Hastie ND. Proc. Natl. Acad. Sci. U.S.A. 89, 4791-5, (1992). View articlePMID: 1317572

GO terms

molecular function

  • None

cellular component

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