WT1

Wilms tumor 1
File:PBB Protein WT1 image.jpg PDB rendering based on 1xf7.
Available structures PDB Ortholog search: Template:Homologene2PDBe PDBe, Template:Homologene2uniprot RCSB List of PDB id codes 1xf7​
Identifiers
Symbols	WT1 ; WAGR; GUD; WIT-2; WT33
External IDs	Template:OMIM5 Template:MGI HomoloGene: 11536
Gene ontology Molecular function Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Cellular component Template:GNF GO Template:GNF GO Template:GNF GO Biological process Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO
RNA expression pattern
File:PBB GE WT1 206067 s at tn.png
File:PBB GE WT1 216953 s at tn.png
More reference expression data
Orthologs
Template:GNF Ortholog box
Species	Human	Mouse
Entrez	n/a	n/a
Ensembl	n/a	n/a
UniProt	n/a	n/a
RefSeq (mRNA)	n/a	n/a
RefSeq (protein)	n/a	n/a
Location (UCSC)	n/a	n/a
PubMed search	n/a	n/a

Wilms tumor 1, also known as WT1, is a human 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 small subset of patients with Wilm's tumors. 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.

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

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. PMID 11595161.CS1 maint: Multiple names: authors list (link)
• 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. PMID 11738793.CS1 maint: Multiple names: authors list (link)
• 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.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Wagner KD, Wagner N, Schedl A (2004). "The complex life of WT1". J. Cell. Sci. 116 (Pt 9): 1653–8. PMID 12665546.CS1 maint: Multiple names: authors list (link)
• Niaudet P, Gubler MC (2007). "WT1 and glomerular diseases". Pediatr. Nephrol. 21 (11): 1653–60. doi:10.1007/s00467-006-0208-1. PMID 16927106.
• 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.