Wilms Tumor Protein (WT1)



Wilms Tumor Protein (WT1)
Wilms tumor protein is 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.WT1 mRNA undergoes RNA editing in human and rat, and that this process is tissue-restricted and developmentally regulated.The WT1 protein has been found to bind a host of cellular factors, e.g. p53, a known tumor suppressor.

Organism species: Homo sapiens (Human)

ProteinsRPF116Hu01Recombinant Wilms Tumor Protein (WT1)Positive Control; Immunogen; SDS-PAGE; WB.
CPF116Hu21OVA Conjugated Wilms Tumor Protein (WT1)Immunogen; SDS-PAGE; WB.
AntibodiesPAF116Hu01Polyclonal Antibody to Wilms Tumor Protein (WT1)WB; IHC; ICC; IP.
MAF116Hu21Monoclonal Antibody to Wilms Tumor Protein (WT1)WB; IHC; ICC; IP.
PAF116Hu08Polyclonal Antibody to Wilms Tumor Protein (WT1)WB; IHC; ICC; IP.
Assay KitsSEF116HuELISA Kit for Wilms Tumor Protein (WT1)Enzyme-linked immunosorbent assay for Antigen Detection.
SCF116HuCLIA Kit for Wilms Tumor Protein (WT1)Chemiluminescent immunoassay for Antigen Detection.

Organism species: Mus musculus (Mouse)

ProteinsCPF116Mu21OVA Conjugated Wilms Tumor Protein (WT1)Immunogen; SDS-PAGE; WB.
AntibodiesPAF116Mu08Polyclonal Antibody to Wilms Tumor Protein (WT1)WB; IHC; ICC; IP.
Assay Kitsn/aCLIA Kit for Wilms Tumor Protein (WT1)CLIA Kit Customized Service Offer
n/aELISA Kit for Wilms Tumor Protein (WT1)ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

ProteinsCPF116Ra21OVA Conjugated Wilms Tumor Protein (WT1)Immunogen; SDS-PAGE; WB.
AntibodiesPAF116Ra08Polyclonal Antibody to Wilms Tumor Protein (WT1)WB; IHC; ICC; IP.
Assay Kitsn/aCLIA Kit for Wilms Tumor Protein (WT1)CLIA Kit Customized Service Offer
n/aELISA Kit for Wilms Tumor Protein (WT1)ELISA Kit Customized Service Offer
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  10. "Germline intronic and exonic mutations in the Wilms' tumour gene (WT1) affecting urogenital development."Nat. Genet. 1:144-148(1992) [PubMed] [Europe PMC] [Abstract]
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  22. "WT1 interacts with the splicing protein RBM4 and regulates its ability to modulate alternative splicing in vivo."Exp. Cell Res. 312:3379-3388(2006) [PubMed] [Europe PMC] [Abstract]
  23. "Contribution of individual amino acids to the RNA binding activity of the Wilms' tumor suppressor protein WT1."Biochemistry 48:148-155(2009) [PubMed] [Europe PMC] [Abstract]
  24. "The tumor suppressor WTX shuttles to the nucleus and modulates WT1 activity."Proc. Natl. Acad. Sci. U.S.A. 106:8338-8343(2009) [PubMed] [Europe PMC] [Abstract]
  25. "Why zinc fingers prefer zinc: ligand-field symmetry and the hidden thermodynamics of metal ion selectivity."Biochemistry 43:13910-13925(2004) [PubMed] [Europe PMC] [Abstract]
  26. "Structure of the Wilms tumor suppressor protein zinc finger domain bound to DNA."J. Mol. Biol. 372:1227-1245(2007) [PubMed] [Europe PMC] [Abstract]
  27. "Zinc finger point mutations within the WT1 gene in Wilms tumor patients."Proc. Natl. Acad. Sci. U.S.A. 89:4791-4795(1992) [PubMed] [Europe PMC] [Abstract]
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  29. "Evidence that WT1 mutations in Denys-Drash syndrome patients may act in a dominant-negative fashion."Hum. Mol. Genet. 2:259-264(1993) [PubMed] [Europe PMC] [Abstract]
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  33. "The Wilms tumour gene WT1 is expressed in murine mesoderm-derived tissues and mutated in a human mesothelioma."Nat. Genet. 4:415-420(1993) [PubMed] [Europe PMC] [Abstract]
  34. "WT1 mutations in patients with Denys-Drash syndrome: a novel mutation in exon 8 and paternal allele origin."Hum. Genet. 93:115-120(1994) [PubMed] [Europe PMC] [Abstract]
  35. "A newly identified exonic mutation of the WT1 gene in a patient with Denys-Drash syndrome."Acta Paediatr. Jpn. Overseas Ed. 38:265-266(1996) [PubMed] [Europe PMC] [Abstract]
  36. "A novel mutation H373Y in the Wilms' tumor suppressor gene, WT1, associated with Denys-Drash syndrome."Hum. Hered. 46:336-338(1996) [PubMed] [Europe PMC] [Abstract]
  37. "Correlation of germ-line mutations and two-hit inactivation of the WT1 gene with Wilms tumors of stromal-predominant histology."Proc. Natl. Acad. Sci. U.S.A. 94:3972-3977(1997) [PubMed] [Europe PMC] [Abstract]
  38. "Identification of constitutional WT1 mutations, in patients with isolated diffuse mesangial sclerosis, and analysis of genotype/phenotype correlations by use of a computerized mutation database."Am. J. Hum. Genet. 62:824-833(1998) [PubMed] [Europe PMC] [Abstract]
  39. "Do intronic mutations affecting splicing of WT1 exon 9 cause Frasier syndrome?"J. Med. Genet. 35:45-48(1998) [PubMed] [Europe PMC] [Abstract]
  40. "Spectrum of early onset nephrotic syndrome associated with WT1 missense mutations."Kidney Int. 53:1594-1600(1998) [PubMed] [Europe PMC] [Abstract]
  41. "Exon 9 mutations in the WT1 gene, without influencing KTS splice isoforms, are also responsible for Frasier syndrome."Hum. Mutat. 14:466-470(1999) [PubMed] [Europe PMC] [Abstract]
  42. "Novel WT1 exon 9 mutation (D396Y) in a patient with early onset Denys Drash syndrome."Hum. Mutat. 15:389-389(2000) [PubMed] [Europe PMC] [Abstract]
  43. "Constitutional WT1 correlate with clinical features in children with progressive nephropathy."J. Med. Genet. 37:698-701(2000) [PubMed] [Europe PMC] [Abstract]
  44. "A novel missense mutation of the WT1 gene causing Denys-Drash syndrome with exceptionally mild renal manifestations."J. Urol. 163:1857-1858(2000) [PubMed] [Europe PMC] [Abstract]
  45. "Novel WT1 mutation (C388Y) in a female child with Denys-Drash syndrome."Pediatr. Nephrol. 16:627-630(2001) [PubMed] [Europe PMC] [Abstract]
  46. "Twenty-four new cases of WT1 germline mutations and review of the literature: genotype/phenotype correlations for Wilms tumor development."Am. J. Med. Genet. A 127:249-257(2004) [PubMed] [Europe PMC] [Abstract]
  47. "Mutation analysis of five candidate genes in Chinese patients with hypospadias."Eur. J. Hum. Genet. 12:706-712(2004) [PubMed] [Europe PMC] [Abstract]
  48. "Prevalence of WT1 mutations in a large cohort of patients with steroid-resistant and steroid-sensitive nephrotic syndrome."Kidney Int. 66:564-570(2004) [PubMed] [Europe PMC] [Abstract]
  49. "A novel mutation of WT1 exon 9 in a patient with Denys-Drash syndrome and pyloric stenosis."Pediatr. Nephrol. 19:1160-1163(2004) [PubMed] [Europe PMC] [Abstract]
  50. "WT1 mutations in Meacham syndrome suggest a coelomic mesothelial origin of the cardiac and diaphragmatic malformations."Am. J. Med. Genet. A 143:2312-2320(2007) [PubMed] [Europe PMC] [Abstract]