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Wilms tumor 1

PDB rendering based on 1xf7.
Available structures
Symbols WT1; WAGR; GUD; WIT-2; WT33
External IDs OMIM607102 MGI98968 HomoloGene11536 GeneCards: WT1 Gene
RNA expression pattern
PBB GE WT1 206067 s at tn.png
PBB GE WT1 216953 s at tn.png
More reference expression data
Species Human Mouse
Entrez 7490 22431
Ensembl ENSG00000184937 n/a
UniProt P19544 n/a
RefSeq (mRNA) NM_000378 XM_001001558
RefSeq (protein) NP_000369 XP_001001558
Location (UCSC) Chr 11:
32.37 - 32.41 Mb
PubMed search [1] [2]

Wilms tumor protein is a protein that in humans is encoded by the WT1 gene.

This gene encodes a transcription factor that contains four zinc-finger motifs at the C-terminus and a proline/glutamine-rich DNA-binding domain at the N-terminus. It has an essential role in the normal development of the urogenital system, and it is mutated in a subset of patients with Wilms' tumor, the gene's namesake. Multiple transcript variants, resulting from alternative splicing at two coding exons, have been well characterized. There is also evidence for the use of non-AUG (CUG) translation initiation site upstream of, and in-frame with the first AUG, leading to additional isoforms.[1]

The WT1 protein has been found to bind a host of cellular factors, e.g. p53, a known tumor suppressor.


WT1 has been shown to interact with U2AF2,[2] PAWR,[3] UBE2I[4] and WTAP.[5]


  1. ^ "Entrez Gene: WT1 Wilms tumor 1".  
  2. ^ Davies, R C; Calvio C, Bratt E, Larsson S H, Lamond A I, Hastie N D (Oct. 1998). "WT1 interacts with the splicing factor U2AF65 in an isoform-dependent manner and can be incorporated into spliceosomes". Genes Dev. (UNITED STATES) 12 (20): 3217–25. ISSN 0890-9369. PMID 9784496.  
  3. ^ Johnstone, R W; See R H, Sells S F, Wang J, Muthukkumar S, Englert C, Haber D A, Licht J D, Sugrue S P, Roberts T, Rangnekar V M, Shi Y (Dec. 1996). "A novel repressor, par-4, modulates transcription and growth suppression functions of the Wilms' tumor suppressor WT1". Mol. Cell. Biol. (UNITED STATES) 16 (12): 6945–56. ISSN 0270-7306. PMID 8943350.  
  4. ^ Wang, Z Y; Qiu Q Q, Seufert W, Taguchi T, Testa J R, Whitmore S A, Callen D F, Welsh D, Shenk T, Deuel T F (Oct. 1996). "Molecular cloning of the cDNA and chromosome localization of the gene for human ubiquitin-conjugating enzyme 9". J. Biol. Chem. (UNITED STATES) 271 (40): 24811–6. ISSN 0021-9258. PMID 8798754.  
  5. ^ Little, N A; Hastie N D, Davies R C (Sep. 2000). "Identification of WTAP, a novel Wilms' tumour 1-associating protein". Hum. Mol. Genet. (ENGLAND) 9 (15): 2231–9. ISSN 0964-6906. PMID 11001926.  

Further reading

  • Haber DA, Buckler AJ (1992). "WT1: a novel tumor suppressor gene inactivated in Wilms' tumor.". New Biol. 4 (2): 97–106. PMID 1313285.  
  • Rauscher FJ (1993). "The WT1 Wilms tumor gene product: a developmentally regulated transcription factor in the kidney that functions as a tumor suppressor.". Faseb J. 7 (10): 896–903. PMID 8393820.  
  • Lee SB, Haber DA (2001). "Wilms tumor and the WT1 gene.". Exp. Cell Res. 264 (1): 74–99. doi:10.1006/excr.2000.5131. PMID 11237525.  
  • Scharnhorst V, van der Eb AJ, Jochemsen AG (2001). "WT1 proteins: functions in growth and differentiation.". Gene 273 (2): 141–61. doi:10.1016/S0378-1119(01)00593-5. PMID 11595161.  
  • Lim HN, Hughes IA, Hawkins JR (2003). "Clinical and molecular evidence for the role of androgens and WT1 in testis descent.". Mol. Cell. Endocrinol. 185 (1-2): 43–50. doi:10.1016/S0303-7207(01)00631-1. PMID 11738793.  
  • Heathcott RW, Morison IM, Gubler MC, et al. (2002). "A review of the phenotypic variation due to the Denys-Drash syndrome-associated germline WT1 mutation R362X.". Hum. Mutat. 19 (4): 462. doi:10.1002/humu.9031. PMID 11933209.  
  • Wagner KD, Wagner N, Schedl A (2004). "The complex life of WT1.". J. Cell. Sci. 116 (Pt 9): 1653–8. doi:10.1242/jcs.00405. PMID 12665546.  
  • Amini Nik S, Hohenstein P (2005). "Upregulation of Wilms' tumor gene 1 (WT1) in desmoid tumors.". Int J Cancer 114 (2): 202–8. doi:10.1002/ijc.20717. PMID 15540161.  
  • Niaudet P, Gubler MC (2007). "WT1 and glomerular diseases.". Pediatr. Nephrol. 21 (11): 1653–60. doi:10.1007/s00467-006-0208-1. PMID 16927106.  
  • Coosemans A, Amini Nik S (2007). "Upregulation of Wilms' tumour gene 1 (WT1) in uterine sarcomas.". Eur J Cancer 43 (10): 1630–37. doi:10.1016/j.ejca.2007.04.008. PMID 17531467.  
  • Hohenstein P, Hastie ND (2006). "The many facets of the Wilms' tumour gene, WT1.". Hum. Mol. Genet. 15 Spec No 2: R196–201. doi:10.1093/hmg/ddl196. PMID 16987884.  


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