Anti-Von Hippel Lindau antibody [52A11] (ab115496)

Overview

• Product nameAnti-Von Hippel Lindau antibody [52A11]See all Von Hippel Lindau primary antibodies ...
• Description
  Mouse monoclonal [52A11] to Von Hippel Lindau
• Tested applicationsFlow Cyt, WB, IHC-P, ELISA more details
• Species reactivity
  Reacts with: Human
  
• Immunogen

  Recombinant fragment, corresponding to amino acids 1-154 of Human Von Hippel Lindau protein with a His tag, purified from E. coli.

• Positive controlHuman Pancreas tissue; HepG2 cell lysate.

Properties

• FormLiquid
• Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze / thaw cycles.
• Storage bufferpH: 7.40
  Preservative: 0.1% Sodium azide
  Constituent: 99% PBS
• Concentration information loading...
• PurityProtein G purified
• Clonality Monoclonal
• Clone number52A11
• IsotypeIgG2b
• Light chain typekappa
• Research Areas
  • Metabolism
  • Types of disease
  • Cancer

  • Metabolism
  • Pathways and Processes
  • Cofactors, Vitamins / minerals
  • Co-factors

  • Metabolism
  • Pathways and Processes
  • Metabolism processes
  • Hypoxia

  • Cancer
  • Oncoproteins/suppressors
  • Tumor suppressors
  • Other

  • Cancer
  • Cell cycle
  • Cell differentiation

  • Cancer
  • Cancer Metabolism
  • Response to hypoxia

  • Epigenetics and Nuclear Signaling
  • Transcription
  • Co-factors

  • Epigenetics and Nuclear Signaling
  • Transcription
  • Polymerase associated factors
  • Pol II Transcription
  • Other

  • Cell Biology
  • Cell Cycle
  • Cell differentiation

  • Cell Biology
  • Proteolysis / Ubiquitin
  • Proteasome / Ubiquitin
  • Ubiquitin

  • Cell Biology
  • Proteolysis / Ubiquitin
  • Proteasome / Ubiquitin
  • Proteasome

  • Cell Biology
  • Cell Cycle
  • Cell Cycle Inhibitors
  • Other

Applications

Target

• FunctionInvolved 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.
• Tissue specificityExpressed in the adult and fetal brain and kidney.
• PathwayProtein modification; protein ubiquitination.
• Involvement in diseaseDefects 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.
• DomainThe Elongin BC complex binding domain is also known as BC-box with the consensus [APST]-L-x(3)-C-x(3)-[AILV].
• Cellular localizationCytoplasm. 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.

Target information above from: UniProt accession P40337 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt
• Database links
  • Entrez Gene: 7428 Human
  • Omim: 608537 Human
  • SwissProt: P40337 Human
  • Unigene: 517792 Human
  • Unigene: 607789 Human

• Alternative names
  Elongin binding protein antibodyG7 protein antibodyHRCA 1 antibody

  HRCA1 antibodyProtein G7 antibodypVHL antibodypVHL antibodyRCA 1 antibodyRCA1 antibodyVHL 1 antibodyVHL antibodyVHL_HUMAN antibodyVHL1 antibodyVHLH antibodyVon Hippel Lindau disease tumor suppressor antibodyvon Hippel Lindau syndrome antibodyvon Hippel Lindau tumor suppressor antibodyVon Hippel Lindau tumor suppressor, E3 ubiquitin protein ligase antibodyVon Hippel-Lindau disease tumor suppressor antibody

  see all

Anti-Von Hippel Lindau antibody [52A11] images

• 

  Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Von Hippel Lindau antibody [52A11] (ab115496)
  ab115496, at 15 µg/ml, staining Von Hippel Lindau in formalin-fixed, paraffin-embedded Human Pancreas tissue by Immunohistochemistry using a biotinylated anti-mouse IgG secondary antibody, alkaline phosphatase-streptavidin and chromogen.
• 

  Western blot - Anti-Von Hippel Lindau antibody [52A11] (ab115496)
  Anti-Von Hippel Lindau antibody [52A11] (ab115496) at 1/500 dilution + HepG2 cell lysate
  
  Secondary
  Goat anti-mouse secondary antibody conjugated to HRP
  developed using the ECL technique
  
  Predicted band size : 24 kDa
  
• 

  Flow Cytometry-Anti-Von Hippel Lindau antibody [52A11](ab115496)
  Overlay histogram showing HepG2 cells stained with ab115496 (red line). The cells were fixed with 4% paraformaldehyde (10 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab115496, 2µg/1x10⁶ cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG2b [PLPV219] (ab91366, 2µg/1x10⁶ cells) used under the same conditions. Acquisition of >5,000 events was performed. This antibody gave a positive signal in HepG2 cells fixed with 80% methanol (5 min)/permeabilized with 0.1% PBS-Tween for 20 min used under the same conditions.