WT1

From Wikipedia, the free encyclopedia

Wilms tumor 1

PDB rendering based on 1xf7.

Available structures: 1xf7

Identifiers

Symbol(s)

WT1; WAGR; GUD; WIT-2; WT33

External IDs

OMIM: 607102 MGI: 98968 HomoloGene: 11536

Gene ontology
Molecular Function:	• nucleic acid binding • transcription factor activity • protein binding • zinc ion binding • metal ion binding
Cellular Component:	• intracellular • nucleus • cytoplasm
Biological Process:	• vasculogenesis • metanephros development • mesonephros development • transcription • regulation of transcription, DNA-dependent • cell cycle • germ cell development • male gonad development • tissue development • epithelial cell differentiation • negative regulation of progression through cell cycle • positive regulation of transcription from RNA polymerase II promoter

RNA expression pattern

More reference expression data

Orthologs

Human

Mouse

n/a

n/a

Refseq

NM_000378 (mRNA)
NP_000369 (protein)

XM_001001558 (mRNA)
XP_001001558 (protein)

Location

Chr 11: 32.37 - 32.41 Mb

n/a

Pubmed search

[1]

[2]

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.^[1]

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

[edit] References

^ Entrez Gene: WT1 Wilms tumor 1.

[edit] 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.
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.
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. PMID 12665546.
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.