Anti-Von Hippel Lindau抗体(ab28434)

概述

  • 产品名称Anti-Von Hippel Lindau抗体
    参阅全部 Von Hippel Lindau 一抗
  • 描述
    兔多克隆抗体to Von Hippel Lindau
  • 特异性ab28434 recognises Von Hippel Lindau protein.
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant full length protein (Human): Von Hippel Lindau protein

  • 阳性对照
    • HeLa nuclear extract

相关产品

应用

Our Abpromise guarantee covers the use of ab28434 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
WB 1/2000 - 1/5000. Predicted molecular weight: 24 kDa.

靶标

  • 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 (ab28434)参考文献

This product has been referenced in:
  • Shmueli MD  et al. Computational and experimental characterization of dVHL establish a Drosophila model of VHL syndrome. PLoS One 9:e109864 (2014). Read more (PubMed: 25310726) »
  • Gammon L  et al. Sub-sets of cancer stem cells differ intrinsically in their patterns of oxygen metabolism. PLoS One 8:e62493 (2013). WB ; Human . Read more (PubMed: 23638097) »

See all 3 Publications for this product

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