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Gene Review

WT2  -  Wilms tumor 2

Homo sapiens

Synonyms: ADCR, MTACR1
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Disease relevance of WT2


Psychiatry related information on WT2


High impact information on WT2

  • These results provide in vivo functional evidence for the existence of a second genetic locus (WT2) involved in suppressing the tumorigenic phenotype of Wilms tumor [7].
  • These data suggest that KIP2 is a BWS gene but that it is not uniquely equivalent to the 11p15.5 "WT2" tumor-suppressor locus [8].
  • WT2 is defined by maternal-specific loss of heterozygosity (LOH) on chromosome 11p15.5 in Wilms' tumors (WTs) [9].
  • Three separate genes (WT1, WT2, and WT3) have been implicated in Wilms' tumour development [10].
  • 5. Here, we have used loss of heterozygosity (LOH) in Wilms' tumors to narrow the WT2 locus distal to the D11S988 locus [11].

Biological context of WT2


Associations of WT2 with chemical compounds

  • O2 deficit decreased between WT1 and WT2 in H only (P < 0.01) [15].
  • Arterialized blood pH and concentrations of bicarbonate ([HCO3-]), venous plasma lactate ([La-]), norepinephrine ([NE]), and epinephrine ([Epi]) were measured before and after WT1 and WT2 [15].
  • The Km for dehydroepiandrosterone and Ki for epostane measured with both the H156Y mutant and WT 2 are 13-fold to 17-fold greater than those values obtained with the WT 1 3beta-HSD [16].

Other interactions of WT2


  1. The role of Wilms' tumor genes. Hirose, M. J. Med. Invest. (1999) [Pubmed]
  2. Novel transcribed sequences within the BWS/WT2 region in 11p15.5: tissue-specific expression correlates with cancer type. Crider-Miller, S.J., Reid, L.H., Higgins, M.J., Nowak, N.J., Shows, T.B., Futreal, P.A., Weissman, B.E. Genomics (1997) [Pubmed]
  3. Loss of heterozygosity at chromosome regions 22q11-12 and 11p15.5 in renal rhabdoid tumors. Schofield, D.E., Beckwith, J.B., Sklar, J. Genes Chromosomes Cancer (1996) [Pubmed]
  4. Mediastinal mass following chemotherapeutic treatment of Hodgkin's disease: recurrent tumor or thymic hyperplasia? Ford, E.G., Lockhart, S.K., Sullivan, M.P., Andrassy, R.J. J. Pediatr. Surg. (1987) [Pubmed]
  5. Magnetic resonance imaging and diffusion-weighted images of cystic meningioma: correlating with histopathology. Chen, T.Y., Lai, P.H., Ho, J.T., Wang, J.S., Chen, W.L., Pan, H.B., Wu, M.T., Chen, C., Liang, H.L., Yang, C.F. Clinical imaging. (2004) [Pubmed]
  6. Molecular genetics of Wilms tumor. Grundy, P., Coppes, M.J., Haber, D. Hematol. Oncol. Clin. North Am. (1995) [Pubmed]
  7. Suppression of tumorigenicity in Wilms tumor by the p15.5-p14 region of chromosome 11. Dowdy, S.F., Fasching, C.L., Araujo, D., Lai, K.M., Livanos, E., Weissman, B.E., Stanbridge, E.J. Science (1991) [Pubmed]
  8. Coding mutations in p57KIP2 are present in some cases of Beckwith-Wiedemann syndrome but are rare or absent in Wilms tumors. O'Keefe, D., Dao, D., Zhao, L., Sanderson, R., Warburton, D., Weiss, L., Anyane-Yeboa, K., Tycko, B. Am. J. Hum. Genet. (1997) [Pubmed]
  9. Multipoint analysis of human chromosome 11p15/mouse distal chromosome 7: inclusion of H19/IGF2 in the minimal WT2 region, gene specificity of H19 silencing in Wilms' tumorigenesis and methylation hyper-dependence of H19 imprinting. Dao, D., Walsh, C.P., Yuan, L., Gorelov, D., Feng, L., Hensle, T., Nisen, P., Yamashiro, D.J., Bestor, T.H., Tycko, B. Hum. Mol. Genet. (1999) [Pubmed]
  10. The molecular pathology of urological malignancies. Dorkin, T.J., Robson, C.N., Neal, D.E. J. Pathol. (1997) [Pubmed]
  11. A common region of loss of heterozygosity in Wilms' tumor and embryonal rhabdomyosarcoma distal to the D11S988 locus on chromosome 11p15.5. Besnard-Guérin, C., Newsham, I., Winqvist, R., Cavenee, W.K. Hum. Genet. (1996) [Pubmed]
  12. A novel imprinted gene, KCNQ1DN, within the WT2 critical region of human chromosome 11p15.5 and its reduced expression in Wilms' tumors. Xin, Z., Soejima, H., Higashimoto, K., Yatsuki, H., Zhu, X., Satoh, Y., Masaki, Z., Kaneko, Y., Jinno, Y., Fukuzawa, R., Hata, J., Mukai, T. J. Biochem. (2000) [Pubmed]
  13. Wilms tumor in a patient with Prader-Willi syndrome. Coppes, M.J., Sohl, H., Teshima, I.E., Mutirangura, A., Ledbetter, D.H., Weksberg, R. J. Pediatr. (1993) [Pubmed]
  14. Genetics of embryonal tumours of childhood: retinoblastoma, Wilms' tumour and neuroblastoma. Brodeur, G.M. Cancer Surv. (1995) [Pubmed]
  15. Recovery processes after repeated supramaximal exercise at the altitude of 4,350 m. Robach, P., Biou, D., Herry, J.P., Deberne, D., Letournel, M., Vaysse, J., Richalet, J.P. J. Appl. Physiol. (1997) [Pubmed]
  16. Differences in substrate and inhibitor kinetics of human type 1 and type 2 3beta-hydroxysteroid dehydrogenase are explained by the type 1 mutant, H156Y. Thomas, J.L., Mason, J.I., Brandt, S., Norris, W. Endocr. Res. (2002) [Pubmed]
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