WT1 Transcription factor that plays an important role in cellular development and cell survival. Recognizes and binds to the DNA sequence 5'-GCG(T/G)GGGCG-3'. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. It has a tumor suppressor as well as an oncogenic role in tumor formation. Function may be isoform-specific: isoforms lacking the KTS motif may act as transcription factors. Isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing. Isoform 1 has lower affinity for DNA, and can bind RNA. Belongs to the EGR C2H2-type zinc-finger protein family. Expressed in the kidney and a subset of hematopoietic cells. 8 alternative splicing and alternative initiation human isoforms have been reported. Note: This description may include information from UniProtKB.

Protein type: C2H2-type zinc finger protein; DNA-binding; Nucleolus; Transcription factor; Tumor suppressor

Chromosomal Location of human Ortholog: 11p13

Cellular Component: cytoplasm; cytosol; nuclear speck; nucleolus; nucleoplasm; nucleus

Molecular Function: C2H2 zinc finger domain binding; DNA-binding transcription activator activity, RNA polymerase II-specific; DNA-binding transcription factor activity; DNA-binding transcription factor activity, RNA polymerase II-specific; double-stranded methylated DNA binding; hemi-methylated DNA-binding; protein binding; RNA binding; RNA polymerase II cis-regulatory region sequence-specific DNA binding; sequence-specific DNA binding; transcription cis-regulatory region binding; zinc ion binding

Biological Process: adrenal cortex formation; adrenal gland development; branching involved in ureteric bud morphogenesis; camera-type eye development; cardiac muscle cell fate commitment; cellular response to cAMP; cellular response to gonadotropin stimulus; diaphragm development; epithelial cell differentiation; germ cell development; glomerular basement membrane development; glomerulus development; gonad development; heart development; kidney development; male genitalia development; male gonad development; mesenchymal to epithelial transition; metanephric epithelium development; metanephric mesenchyme development; metanephric S-shaped body morphogenesis; negative regulation of apoptotic process; negative regulation of cell growth; negative regulation of cell population proliferation; negative regulation of DNA-templated transcription; negative regulation of female gonad development; negative regulation of metanephric glomerular mesangial cell proliferation; negative regulation of transcription by RNA polymerase II; podocyte differentiation; positive regulation of DNA methylation; positive regulation of DNA-templated transcription; positive regulation of gene expression; positive regulation of heart growth; positive regulation of male gonad development; positive regulation of metanephric ureteric bud development; positive regulation of miRNA transcription; positive regulation of transcription by RNA polymerase II; posterior mesonephric tubule development; regulation of animal organ formation; regulation of DNA-templated transcription; regulation of transcription by RNA polymerase II; RNA splicing; sex determination; thorax and anterior abdomen determination; tissue development; ureteric bud development; vasculogenesis; visceral serous pericardium development

Disease: Aniridia; Denys-drash Syndrome; Frasier Syndrome; Meacham Syndrome; Mesothelioma, Malignant; Nephrotic Syndrome, Type 4; Wilms Tumor 1; Wilms Tumor, Aniridia, Genitourinary Anomalies, And Mental Retardation Syndrome

Reference #: P19544 (UniProtKB)

Alt. Names/Synonyms: AWT1; GUD; NPHS4; WAGR; Wilms tumor 1; Wilms tumor protein; WIT-2; WT1; WT1 transcription factor; WT33

Gene Symbols: WT1

Molecular weight: 49,188 Da

Basal Isoelectric point: 9.23 Predict pI for various phosphorylation states

CST Pathways: ErbB/HER Signaling

Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology^®

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WT1

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