Anti-Von Hippel Lindau抗体[3F391] (ab11189)


  • 产品名称Anti-Von Hippel Lindau抗体[3F391]
    参阅全部 Von Hippel Lindau 一抗
  • 描述
    小鼠单克隆抗体[3F391] to Von Hippel Lindau
  • 特异性Does not recognize the truncated form of VHL protein.
  • 经测试应用适用于: IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant full length protein (Human).

  • 表位Amino acids 1-54 of human VHL protein.
  • 阳性对照
    • Kidney.



Our Abpromise guarantee covers the use of ab11189 in the following tested applications.

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

应用 Ab评论 说明
IHC-P Use a concentration of 2 - 4 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.


  • 功能Involved in the ubiquitination and subsequent proteasomal degradation via the von Hippel-Lindau ubiquitination complex. Seems to act as target recruitment subunit in the E3 ubiquitin ligase complex and recruits hydroxylated hypoxia-inducible factor (HIF) under normoxic conditions. Involved in transcriptional repression through interaction with HIF1A, HIF1AN and histone deacetylases.
  • 组织特异性Expressed in the adult and fetal brain and kidney.
  • 通路Protein modification; protein ubiquitination.
  • 疾病相关Defects in VHL are a cause of susceptibility to pheochromocytoma (PCC) [MIM:171300]. A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent.
    Defects in VHL are the cause of von Hippel-Lindau disease (VHLD) [MIM:193300]. VHLD is a dominantly inherited familial cancer syndrome characterized by the development of retinal angiomatosis, cerebellar and spinal hemangioblastoma, renal cell carcinoma (RCC), phaeochromocytoma and pancreatic tumors. VHL type 1 is without pheochromocytoma, type 2 is with pheochromocytoma. VHL type 2 is further subdivided into types 2A (pheochromocytoma, retinal angioma, and hemangioblastomas without renal cell carcinoma and pancreatic cyst) and 2B (pheochromocytoma, retinal angioma, and hemangioblastomas with renal cell carcinoma and pancreatic cyst). VHL type 2C refers to patients with isolated pheochromocytoma without hemangioblastoma or renal cell carcinoma. The estimated incidence is 3/100000 births per year and penetrance is 97% by age 60 years.
    Defects in VHL are the cause of erythrocytosis familial type 2 (ECYT2) [MIM:263400]; also called VHL-dependent polycythemia or Chuvash type polycythemia. ECYT2 is an autosomal recessive disorder characterized by an increase in serum red blood cell mass, hypersensitivity of erythroid progenitors to erythropoietin, increased erythropoietin serum levels, and normal oxygen affinity. Patients with ECYT2 carry a high risk for peripheral thrombosis and cerebrovascular events.
    Defects in VHL are a cause of renal cell carcinoma (RCC) [MIM:144700]. Renal cell carcinoma is a heterogeneous group of sporadic or hereditary carcinoma derived from cells of the proximal renal tubular epithelium. It is subclassified into clear cell renal carcinoma (non-papillary carcinoma), papillary renal cell carcinoma, chromophobe renal cell carcinoma, collecting duct carcinoma with medullary carcinoma of the kidney, and unclassified renal cell carcinoma.
  • 结构域The Elongin BC complex binding domain is also known as BC-box with the consensus [APST]-L-x(3)-C-x(3)-[AILV].
  • 细胞定位Cytoplasm. Membrane. Nucleus. Found predominantly in the cytoplasm and with less amounts nuclear or membrane-associated and Cytoplasm. Nucleus. Equally distributed between the nucleus and the cytoplasm but not membrane-associated.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Elongin binding protein antibody
    • G7 protein antibody
    • HRCA 1 antibody
    • HRCA1 antibody
    • Protein G7 antibody
    • pVHL antibody
    • RCA 1 antibody
    • RCA1 antibody
    • VHL 1 antibody
    • VHL antibody
    • VHL_HUMAN antibody
    • VHL1 antibody
    • VHLH antibody
    • Von Hippel Lindau disease tumor suppressor antibody
    • von Hippel Lindau syndrome antibody
    • von Hippel Lindau tumor suppressor antibody
    • Von Hippel Lindau tumor suppressor, E3 ubiquitin protein ligase antibody
    • Von Hippel-Lindau disease tumor suppressor antibody
    see all

Anti-Von Hippel Lindau antibody [3F391] (ab11189)参考文献

This product has been referenced in:
  • Gimelli S  et al. The tumor suppressor gene TRC8/RNF139 is disrupted by a constitutional balanced translocation t(8;22)(q24.13;q11.21) in a young girl with dysgerminoma. Mol Cancer 8:52 (2009). IHC-P ; Human . Read more (PubMed: 19642973) »
  • Hacker KE  et al. VHL type 2B mutations retain VBC complex form and function. PLoS ONE 3:e3801 (2008). WB ; Human . Read more (PubMed: 19030229) »

See all 3 Publications for this product

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Abcam has not validated the combination of species/application used in this Abreview.
Application Western blot
Sample Human Cell lysate - whole cell (placenta)
Specification placenta
Blocking step Milk as blocking agent for 3 hour(s) and 0 minute(s) · Concentration: 5%

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提交于 Aug 12 2005