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Gene Review

WT1  -  Wilms tumor 1

Homo sapiens

Synonyms: AWT1, EWS-WT1, GUD, NPHS4, WAGR, ...
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Disease relevance of WT1


Psychiatry related information on WT1


High impact information on WT1


Chemical compound and disease context of WT1


Biological context of WT1

  • The Wilms tumor suppressor gene WT1 encodes a developmentally regulated transcription factor that is mutated in a subset of embryonal tumors [1].
  • Induction of WT1 resulted in programmed cell death [1].
  • This effect, which was differentially mediated by the alternative splicing variants of WT1, was independent of p53 [1].
  • The activity of WT1 varied significantly depending on the presence or absence of an alternatively spliced region located upstream of the zinc finger domain [18].
  • We now demonstrate that p53, the product of a tumor-suppressor gene with ubiquitous expression, physically associates with WT1 in transfected cells [19].

Anatomical context of WT1


Associations of WT1 with chemical compounds


Physical interactions of WT1


Regulatory relationships of WT1

  • WT1 splice variants lacking a KTS insert between zinc fingers 3 and 4 suppressed IGF-IR promoter activity in the absence of p53 or in the presence of wild-type p53 [28].
  • Functionally, BMZF2 inhibits transcriptional activation by WT1 [23].
  • PODXL is also transcriptionally regulated by WT1 and has roles in cell adhesion and anti-adhesion [29].
  • To examine the potential interplay between WT1 and ERalpha in control of IGF-IR gene transcription we employed ER-depleted C4 cells that were generated by clonal selection of ER-positive MCF-7 cells that were maintained in estrogen-free conditions [25].
  • Taken together, our results show that BASP1 is a WT1-associated factor that can regulate WT1 transcriptional activity [2].

Other interactions of WT1


Analytical, diagnostic and therapeutic context of WT1

  • Microinjection of the WT1 cDNA into quiescent cells or cells in early to mid G1 phase blocked serum-induced cell cycle progression into S phase [18].
  • RT-PCR analysis showed that the mean values of WT1 RNA were 3413, 712, and 334 copies/microg RNA in PNH, AA, and healthy individuals, respectively [4].
  • Co-immunoprecipitation experiments are consistent with an in vivo association between WT1 and BMZF2 [23].
  • Electrophoretic mobility shift assays showed strong binding by recombinant WT1(-)KTS protein and weaker or no binding by the WT1(+)KTS protein to DNA probes containing WT1 binding sites from these three promoters [30].
  • We suggest that the molecular techniques used here (heterozygosity testing for polymorphic markers mapping between AN2 and WT1 and deletion analysis by dosage, cytogenetic analysis, or in situ hybridization) can be employed to identify sporadic aniridia patients with and without increased tumor risk [31].


  1. WT1 suppresses synthesis of the epidermal growth factor receptor and induces apoptosis. Englert, C., Hou, X., Maheswaran, S., Bennett, P., Ngwu, C., Re, G.G., Garvin, A.J., Rosner, M.R., Haber, D.A. EMBO J. (1995) [Pubmed]
  2. BASP1 is a transcriptional cosuppressor for the Wilms' tumor suppressor protein WT1. Carpenter, B., Hill, K.J., Charalambous, M., Wagner, K.J., Lahiri, D., James, D.I., Andersen, J.S., Schumacher, V., Royer-Pokora, B., Mann, M., Ward, A., Roberts, S.G. Mol. Cell. Biol. (2004) [Pubmed]
  3. WT1 is a key regulator of podocyte function: reduced expression levels cause crescentic glomerulonephritis and mesangial sclerosis. Guo, J.K., Menke, A.L., Gubler, M.C., Clarke, A.R., Harrison, D., Hammes, A., Hastie, N.D., Schedl, A. Hum. Mol. Genet. (2002) [Pubmed]
  4. HLA class II haplotype and quantitation of WT1 RNA in Japanese patients with paroxysmal nocturnal hemoglobinuria. Shichishima, T., Okamoto, M., Ikeda, K., Kaneshige, T., Sugiyama, H., Terasawa, T., Osumi, K., Maruyama, Y. Blood (2002) [Pubmed]
  5. Transcriptional activity of testis-determining factor SRY is modulated by the Wilms' tumor 1 gene product, WT1. Matsuzawa-Watanabe, Y., Inoue, J., Semba, K. Oncogene (2003) [Pubmed]
  6. The transcription factor Wilms tumor 1 regulates matrix metalloproteinase-9 through a nitric oxide-mediated pathway. Marcet-Palacios, M., Ulanova, M., Duta, F., Puttagunta, L., Munoz, S., Gibbings, D., Radomski, M., Cameron, L., Mayers, I., Befus, A.D. J. Immunol. (2007) [Pubmed]
  7. Wilms' tumor suppressor (WT1) is a mediator of neuronal degeneration associated with the pathogenesis of Alzheimer's disease. Lovell, M.A., Xie, C., Xiong, S., Markesbery, W.R. Brain Res. (2003) [Pubmed]
  8. Submicroscopic deletions at the WAGR locus, revealed by nonradioactive in situ hybridization. Fantes, J.A., Bickmore, W.A., Fletcher, J.M., Ballesta, F., Hanson, I.M., van Heyningen, V. Am. J. Hum. Genet. (1992) [Pubmed]
  9. Expression of steroidogenic factor 1 and Wilms' tumour 1 during early human gonadal development and sex determination. Hanley, N.A., Ball, S.G., Clement-Jones, M., Hagan, D.M., Strachan, T., Lindsay, S., Robson, S., Ostrer, H., Parker, K.L., Wilson, D.I. Mech. Dev. (1999) [Pubmed]
  10. Contralateral intralobar nephrogenic rests: possible confusion with bilateral Wilms tumor. Baskin, L.S., Massad, C.A., Cohen, M.B., Ablin, A.R., Kogan, B.A. J. Urol. (1991) [Pubmed]
  11. Antithrombin treatment of severe hepatic veno-occlusive disease in children with cancer. Mertens, R., Brost, H., Granzen, B., Nowak-Göttl, U. Eur. J. Pediatr. (1999) [Pubmed]
  12. The Wilms tumor suppressor WT1 encodes a transcriptional activator of amphiregulin. Lee, S.B., Huang, K., Palmer, R., Truong, V.B., Herzlinger, D., Kolquist, K.A., Wong, J., Paulding, C., Yoon, S.K., Gerald, W., Oliner, J.D., Haber, D.A. Cell (1999) [Pubmed]
  13. Wilms' tumor 1 and Dax-1 modulate the orphan nuclear receptor SF-1 in sex-specific gene expression. Nachtigal, M.W., Hirokawa, Y., Enyeart-VanHouten, D.L., Flanagan, J.N., Hammer, G.D., Ingraham, H.A. Cell (1998) [Pubmed]
  14. Prevalence of WT1 mutations in a large cohort of patients with steroid-resistant and steroid-sensitive nephrotic syndrome. Ruf, R.G., Schultheiss, M., Lichtenberger, A., Karle, S.M., Zalewski, I., Mucha, B., Everding, A.S., Neuhaus, T., Patzer, L., Plank, C., Haas, J.P., Ozaltin, F., Imm, A., Fuchshuber, A., Bakkaloglu, A., Hildebrandt, F. Kidney Int. (2004) [Pubmed]
  15. Expression of the Wilms' tumor gene WT1 in human malignant mesothelioma cell lines and relationship to platelet-derived growth factor A and insulin-like growth factor 2 expression. Langerak, A.W., Williamson, K.A., Miyagawa, K., Hagemeijer, A., Versnel, M.A., Hastie, N.D. Genes Chromosomes Cancer (1995) [Pubmed]
  16. Differential regulation of the Wilms' tumor gene, WT1, during differentiation of embryonal carcinoma and embryonic stem cells. Scharnhorst, V., Kranenburg, O., van der Eb, A.J., Jochemsen, A.G. Cell Growth Differ. (1997) [Pubmed]
  17. Downregulation of Wilms' tumor 1 protein inhibits breast cancer proliferation. Zapata-Benavides, P., Tuna, M., Lopez-Berestein, G., Tari, A.M. Biochem. Biophys. Res. Commun. (2002) [Pubmed]
  18. G1 phase arrest induced by Wilms tumor protein WT1 is abrogated by cyclin/CDK complexes. Kudoh, T., Ishidate, T., Moriyama, M., Toyoshima, K., Akiyama, T. Proc. Natl. Acad. Sci. U.S.A. (1995) [Pubmed]
  19. Physical and functional interaction between WT1 and p53 proteins. Maheswaran, S., Park, S., Bernard, A., Morris, J.F., Rauscher, F.J., Hill, D.E., Haber, D.A. Proc. Natl. Acad. Sci. U.S.A. (1993) [Pubmed]
  20. WT1 mutation in malignant mesothelioma and WT1 immunoreactivity in relation to p53 and growth factor receptor expression, cell-type transition, and prognosis. Kumar-Singh, S., Segers, K., Rodeck, U., Backhovens, H., Bogers, J., Weyler, J., Van Broeckhoven, C., Van Marck, E. J. Pathol. (1997) [Pubmed]
  21. WT1 and DAX-1 inhibit aromatase P450 expression in human endometrial and endometriotic stromal cells. Gurates, B., Sebastian, S., Yang, S., Zhou, J., Tamura, M., Fang, Z., Suzuki, T., Sasano, H., Bulun, S.E. J. Clin. Endocrinol. Metab. (2002) [Pubmed]
  22. Role of Wilms tumor 1 (WT1) in the transcriptional regulation of the Mullerian-inhibiting substance promoter. Hossain, A., Saunders, G.F. Biol. Reprod. (2003) [Pubmed]
  23. Inhibition of Wilms tumor 1 transactivation by bone marrow zinc finger 2, a novel transcriptional repressor. Lee, T.H., Lwu, S., Kim, J., Pelletier, J. J. Biol. Chem. (2002) [Pubmed]
  24. WT1 activates a glomerular-specific enhancer identified from the human nephrin gene. Guo, G., Morrison, D.J., Licht, J.D., Quaggin, S.E. J. Am. Soc. Nephrol. (2004) [Pubmed]
  25. The WT1 Wilms' tumor suppressor gene product interacts with estrogen receptor-alpha and regulates IGF-I receptor gene transcription in breast cancer cells. Reizner, N., Maor, S., Sarfstein, R., Abramovitch, S., Welshons, W.V., Curran, E.M., Lee, A.V., Werner, H. J. Mol. Endocrinol. (2005) [Pubmed]
  26. Significant co-expression of WT1 and MDR1 genes in acute myeloid leukemia patients at diagnosis. Galimberti, S., Guerrini, F., Carulli, G., Fazzi, R., Palumbo, G.A., Morabito, F., Petrini, M. Eur. J. Haematol. (2004) [Pubmed]
  27. WT1 interacts with the splicing factor U2AF65 in an isoform-dependent manner and can be incorporated into spliceosomes. Davies, R.C., Calvio, C., Bratt, E., Larsson, S.H., Lamond, A.I., Hastie, N.D. Genes Dev. (1998) [Pubmed]
  28. WT1-p53 interactions in insulin-like growth factor-I receptor gene regulation. Idelman, G., Glaser, T., Roberts, C.T., Werner, H. J. Biol. Chem. (2003) [Pubmed]
  29. The Wilms tumor suppressor-1 target gene podocalyxin is transcriptionally repressed by p53. Stanhope-Baker, P., Kessler, P.M., Li, W., Agarwal, M.L., Williams, B.R. J. Biol. Chem. (2004) [Pubmed]
  30. The Wilms' tumor gene WT1 can regulate genes involved in sex determination and differentiation: SRY, Müllerian-inhibiting substance, and the androgen receptor. Shimamura, R., Fraizer, G.C., Trapman, J., Lau YfC, n.u.l.l., Saunders, G.F. Clin. Cancer Res. (1997) [Pubmed]
  31. The distal region of 11p13 and associated genetic diseases. Mannens, M., Hoovers, J.M., Bleeker-Wagemakers, E.M., Redeker, E., Bliek, J., Overbeeke-Melkert, M., Saunders, G., Williams, B., van Heyningen, V., Junien, C. Genomics (1991) [Pubmed]
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