catalog number :
MBS057373
products type :
ELISA Kit
products full name :
Horse Wilms Tumor Protein ELISA Kit
products short name :
Wilms Tumor Protein
other names :
Wilms tumor protein isoform B; Wilms tumor protein; Wilms tumor protein; amino-terminal domain of EWS last three zinc fingers of the DNA-binding domain of WT1; Wilms tumor 1; WT33
other gene names :
WT1; WT1; GUD; AWT1; WAGR; WT33; NPHS4; WIT-2; EWS-WT1
uniprot entry name :
WT1_HUMAN
storage stability :
Store all reagents at 2-8 degree C
ncbi acc num :
NP_077742.2
ncbi gb acc num :
NM_024424.3
ncbi mol weight :
49,188 Da
ncbi pathways :
Integrated Pancreatic Cancer Pathway (711360); Regulation Of Telomerase Pathway (137987); Transcriptional Misregulation In Cancer Pathway (523016); Transcriptional Misregulation In Cancer Pathway (522987)
ncbi summary :
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. This gene exhibits complex tissue-specific and polymorphic imprinting pattern, with biallelic, and monoallelic expression from the maternal and paternal alleles in different tissues. Multiple transcript variants have been described. In several variants, there is evidence for the use of a non-AUG (CUG) translation initiation site upstream of and in-frame with the first AUG. Authors of PMID:7926762 also provide evidence that WT1 mRNA undergoes RNA editing in human and rat, and that this process is tissue-restricted and developmentally regulated. [provided by RefSeq, Oct 2010]
uniprot summary :
WT1: a DNA binding protein and apparent transcriptional regulator. Recognizes and binds to the DNA sequence 5 -CGCCCCCGC-3 . Expressed in the kidney and a subset of hematopoietic cells. Defects in WT1 are the cause of Wilms tumor 1 (WT1), an embryonal malignancy of the kidney that affects approximately 1 in 10 000 infants and young children. It occurs both in sporadic and hereditary forms. Four alternatively spliced isoforms have been described. Protein type: DNA-binding; Tumor suppressor; Nucleolus; Transcription factor; C2H2-type zinc finger protein. Chromosomal Location of Human Ortholog: 11p13. Cellular Component: nucleoplasm; cytoplasm; nucleolus; nuclear speck; nucleus. Molecular Function: protein binding; zinc ion binding; RNA binding; sequence-specific DNA binding; transcription factor activity. Biological Process: tissue development; gonad development; positive regulation of apoptosis; heart development; positive regulation of transcription, DNA-dependent; thorax and anterior abdomen determination; negative regulation of transcription from RNA polymerase II promoter; glomerulus development; germ cell development; negative regulation of cell proliferation; regulation of transcription, DNA-dependent; epithelial cell differentiation; ureteric bud development; male genitalia development; kidney development; vasculogenesis; camera-type eye development; transcription, DNA-dependent; adrenal gland development; RNA splicing; glomerular basement membrane development; male gonad development; regulation of transcription from RNA polymerase II promoter; sex determination; ureteric bud branching; negative regulation of translation; positive regulation of transcription from RNA polymerase II promoter; negative regulation of cell growth; negative regulation of transcription, DNA-dependent; negative regulation of apoptosis. Disease: Wilms Tumor, Aniridia, Genitourinary Anomalies, And Mental Retardation Syndrome; Denys-drash Syndrome; Mesothelioma, Malignant; Nephrotic Syndrome, Type 4; Frasier Syndrome; Aniridia; Wilms Tumor 1; Meacham Syndrome