货号：05-753

品牌：Merck Millipore

规格：200Ug

目录价：￥5053.00

市场价格：￥4295.05

会员价格：￥4042.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-WT1 Antibody, clone 6F-H2 | 05-753 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Upstate (Millipore) View All» Specificity: WT1 View All» Molecular Weight: 55-60kDa View All» Immunogen: 6His-tagged fusion protein corresponding to residues 1-181 of human WT1 (Wilms tumor) View All» Isotype: IgG1 View All» Species Reactivity: Human View All» Quality Assurance: routinely evaluated by immunoblot on RIPA lysates from Jurkat and Raji cells View All» Purification Method: Protein G purfied View All» Presentation: 0.1M Tris-glycine, pH 7.4, 0.15M NaCl, 0.05% sodium azide before the addition of glycerol to 30% View All» Storage Conditions: 2 years at -20°C View All» UniProt Number: P19544 View All» Entrez Gene Number: NM_024426 View All» Gene Symbol:

• WT1

• WAGR

• GUD

• WT33

• WIT-2
  View All» Sales Restrictions: Due to license agreement restrictions, this product cannot be purchased for resale. View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
  • Western Blotting

  • Immunocytochemistry

  • Immunohistochemistry
    View All» Entrez Gene 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. 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. 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. View All» UniProt Summary: FUNCTION: SwissProt: P19544 # Potential role in transcriptional regulation. Recognizes and binds to the DNA sequence 5'-CGCCCCCGC-3'.SIZE: 449 amino acids; 49188 Da SUBUNIT: Interacts with ZNF224 via the zinc-finger region. Interacts with WTAP and SRY.SUBCELLULAR LOCATION: Nucleus.TISSUE SPECIFICITY: Expressed in the kidney and a subset of hematopoietic cells.DISEASE: SwissProt: P19544 # 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 the genitourinary dysplasia component of WAGR syndrome (GUD) [MIM:137357]. WAGR syndrome is a contiguous gene syndrome characterized by Wilms tumor, aniridia, genitourinary anomalies and mental retardation. These clinical features are due to defects in different genes. & 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 isolated diffuse mesangial sclerosis (IDMS) [MIM:256370]. IDMS is an early-onset nephrotic syndrome occurring in the absence of other abnormalities and resulting in renal failure. Inheritance is autosomal recessive. & A chromosomal aberration involving WT1 may be a cause of desmoplastic small round cell tumor (DSRCT). Translocation t(11;22)(p13;q12) with EWSR1.SIMILARITY: SwissProt: P19544 ## Belongs to the EGR C2H2-type zinc-finger protein family. & Contains 4 C2H2-type zinc fingers. View All» Brand Family: Upstate View All» Product Name: Anti-WT1 Antibody, clone 6F-H2 | 05-753 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 200 µg View All» Format: Purified View All» Host: Mouse View All»
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