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Product Name WT1 Chinese Name 肾母细胞瘤蛋白单克隆抗体 Alias WT1_HUMAN; Wilms tumor protein; WT33; WT1 transcription factor; GUD; AWT1; WAGR; WT-1; WT33; NPHS4; WIT-2; Immunogen Species Mouse Clonality Monoclonal Clone NO. 7B8 React Species (predicted: Human, Mouse, Rat, ) Applications
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 55kDa Cellular localization The nucleus cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human WT1 Lsotype IgG2b/Kappa Purification affinity purified by Protein A Buffer Solution Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail Transcription factor that plays an important role in cellular development and cell survival. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. Recognizes and binds to the DNA sequence 5'-CGCCCCCGC-3'. 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.
Function:
Transcription factor that plays an important role in cellular development and cell survival. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. Recognizes and binds to the DNA sequence 5'-CGCCCCCGC-3'. 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.
Subunit:
Homodimer. Interacts with WTIP. Interacts with actively translating polysomes. Detected in nuclear ribonucleoprotein (mRNP) particles. Interacts with HNRNPU via the zinc-finger region. Interacts with U2AF2. Interacts with CITED2. Interacts with ZNF224 via the zinc-finger region. Interacts with WTAP and SRY. Interacts with FAM123B/WTX. Interacts with RBM4.
Subcellular Location:
Nucleus. Nucleus, nucleolus. Cytoplasm. Note=Shuttles between nucleus and cytoplasm. Isoform 1: Nucleus speckle. Isoform 4: Nucleus, nucleoplasm.
Tissue Specificity:
Expressed in the kidney and a subset of hematopoietic cells.
DISEASE:
Defects in WT1 are the cause of Frasier syndrome (FS) [MIM:136680]. FS is characterized by a slowly progressing nephropathy leading to renal failure in adolescence or early adulthood, male pseudohermaphroditism, and no Wilms tumor. As for histological findings of the kidneys, focal glomerular sclerosis is often observed. There is phenotypic overlap with Denys-Drash syndrome. Inheritance is autosomal dominant. Defects in WT1 are the cause of Wilms tumor 1 (WT1) [MIM:194070]. WT is 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. Defects in WT1 are the cause of Denys-Drash syndrome (DDS) [MIM:194080]. DDS is a typical nephropathy characterized by diffuse mesangial sclerosis, genital abnormalities, and/or Wilms tumor. There is phenotypic overlap with WAGR syndrome and Frasier syndrome. Inheritance is autosomal dominant, but most cases are sporadic.
Similarity:
Belongs to the EGR C2H2-type zinc-finger protein family. Contains 4 C2H2-type zinc fingers.
SWISS:
P19544
Gene ID:
7490
Database links:Entrez Gene: 7490 Human
Entrez Gene: 22431 Mouse
Omim: 607102 Human
SwissProt: P19544 Human
SwissProt: P22561 Mouse
Unigene: 591980 Human
Unigene: 389339 Mouse
Unigene: 92531 Rat
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