Recombinant Human Wilms Tumor Protein (ab83942)

Overview

Description

  • NatureRecombinant
  • SourceBaculovirus
  • Amino Acid Sequence
    • AccessionNM_000378
    • SpeciesHuman
    • Molecular weight56 kDa
    • Amino acids1 to 446
    • TagsDDDDK tag N-Terminus
    • Additional sequence informationIncludes exon 5 but without KTS.

Specifications

Our Abpromise guarantee covers the use of ab83942 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    SDS-PAGE

    Functional Studies

    EMSA

  • Purity>95% by SDS-PAGE .

  • FormLiquid
  • Additional notes

    WT1 without KTS appears to have different binding affinity to both DNA and RNA compared to the one with KTS.

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    Preservative: None
    Constituents: 20% Glycerol, 20mM Tris HCl, 100mM Potassium chloride, 1mM DTT, 0.1mM EDTA, pH 8

General Info

  • Alternative names
    • WIT 2
    • WT 1
    • AWT1
    • FWT1
    • GUD
    • NPHS4
    • WAGR
    • Wilms Tumor
    • Wilms tumor 1
    • Wilms tumor protein
    • Wilms' tumor gene
    • Wilms' tumor protein
    • WIT2
    • WT
    • WT1
    • WT1_HUMAN
    • WT33
    see all
  • FunctionTranscription 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.
  • Tissue specificityExpressed in the kidney and a subset of hematopoietic cells.
  • Involvement in diseaseDefects 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.
    Defects in WT1 are the cause of nephrotic syndrome type 4 (NPHS4) [MIM:256370]. A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. Most patients with NPHS4 show diffuse mesangial sclerosis on renal biopsy, which is a pathologic entity characterized by mesangial matrix expansion with no mesangial hypercellularity, hypertrophy of the podocytes, vacuolized podocytes, thickened basement membranes, and diminished patency of the capillary lumen.
    Defects in WT1 are a cause of Meacham syndrome (MEACHS) [MIM:608978]. Meacham syndrome is a rare sporadically occurring multiple malformation syndrome characterized by male pseudohermaphroditism with abnormal internal female genitalia comprising a uterus and double or septate vagina, complex congenital heart defect and diaphragmatic abnormalities.
    Note=A chromosomal aberration involving WT1 may be a cause of desmoplastic small round cell tumor (DSRCT). Translocation t(11;22)(p13;q12) with EWSR1.
  • Sequence similaritiesBelongs to the EGR C2H2-type zinc-finger protein family.
    Contains 4 C2H2-type zinc fingers.
  • Cellular localizationNucleus. Cytoplasm. Shuttles between nucleus and cytoplasm; Nucleus > nucleoplasm and Nucleus speckle.
  • Information by UniProt

References for Recombinant Human Wilms Tumor Protein (ab83942)

ab83942 has not yet been referenced specifically in any publications.

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