merck millipore,默克密理愽
  • merck millipore,默克密理博,MAB4234,Anti-Wilms' Tumor Antibody, NT, clone 6F-H2

  • merck millipore,默克密理博,MAB4234,Anti-Wilms' Tumor Antibody, NT, clone 6F-H2

    产品名称：Anti-Wilms' Tumor Antibody, NT, clone 6F-H2
    产品型号：MAB4234
    Anti-Wilms' Tumor Antibody, N-terminus, clone 6F-H2 is a Mouse Monoclonal Antibody for detection of Wilms' Tumor also known as WT1 & has been validated in ELISA, IF & WB.

    merck millipore,默克密理博,MAB4234,Anti-Wilms' Tumor Antibody, NT, clone 6F-H2

  • 产品介绍
  • merck millipore,默克密理博,MAB4234,Anti-Wilms' Tumor Antibody, NT, clone 6F-H2

    重要规格表

    品种反应性 主要应用 宿主 格式 抗体类型
    HWB, ELISAMPurifiedMonoclonal Antibody
    描述
    产品目录编号 MAB4234
    品牌系列 Chemicon®
    商名
    • Chemicon
    描述 Anti-Wilms' Tumor Antibody, NT, clone 6F-H2
    Alternate Names
    • WT1
    背景信息 Wilm's Tumor (WT), a sporadic and familial childhood kidney tumor, is genetically heterogeneous. Wilms' tumor is associated with mutations of WT1, a zinc-finger transcription factor that is essential for the development of the metanephric kidney and the urogenital system. The WT1 gene is normally expressed in fetal kidney and mesothelium, and its expression has been suggested as a marker for Wilms tumor and mesothelioma.
    产品信息
    格式 Purified
    演示 Purified immunoglobulin from Protein A Sepharose™ chromatography. Liquid in 0.02M PB, pH 7.6, 0.25M NaCl containing 0.1% sodium azide.
    应用
    应用 Anti-Wilms' Tumor Antibody, N-terminus, clone 6F-H2 is a Mouse Monoclonal Antibody for detection of Wilms' Tumor also known as WT1 & has been validated in ELISA, IF & WB.
    主要应用
    • Western Blotting
    • ELISA
    应用说明 Immunofluorescence:

    Western Blot:

    ELISA:

    Optimal working dilutions must be determined by end user.
    生物信息
    免疫原品种 aa 1-173 amino acides of the WT-1 protein
    表位 N-terminus, nephroblastoma tumor
    克隆 6F-H2
    浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration.
    宿主 Mouse
    特异性 The antibody reacts with all isoforms of the full-length WT1 protein. The antibody staining location is nuclear.
    同种型 IgG1κ
    品种反应性 Human
    抗体类型 Monoclonal Antibody
    Entrez基因编号
    • NM_024426.3
    • NM_000378.3
    • NM_024424.2
    • NM_024425.2
    Entrez基因汇总 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.
    基因符号
    • WIT-2
    • WT1
    • GUD
    • WT33
    • WAGR
    UniProt编号
    • P19544
    UniProt汇总 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.
    产品使用声明
    使用声明
    • 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.
    储存和货运信息
    存储条件 Maintain at 2-8°C in undiluted aliquots for up to 6 months after date of receipt.
    包装信息
    数量 100 µg

    merck millipore,默克密理博,MAB4234,Anti-Wilms' Tumor Antibody, NT, clone 6F-H2

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