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Product name
Anti-Von Hippel Lindau antibody
See all Von Hippel Lindau products (12) ...
Description
Rabbit polyclonal to Von Hippel Lindau
Specificity
ab28434 recognises Von Hippel Lindau protein.
Tested applications
Cross reactivity
Reacts with
Human
Immunogen
Recombinant full length protein (Human): Von Hippel Lindau protein
Positive control
HeLa nuclear extract
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Concentration
Concentration information loading...
Purity
Protein A purified
Clonality
Polyclonal
Isotype
IgG
Research areas
Metabolism >> Types of disease >> Cancer
Metabolism >> Pathways and Processes >> Metabolism processes >> Hypoxia
Metabolism >> Pathways and Processes >> Cofactors, Vitamins / minerals >> Co-factors
Cancer >> Cancer Metabolism >> Response to hypoxia
Cancer >> Oncoproteins/suppressors >> Tumor suppressors >> Other
Cancer >> Cell cycle >> Cell differentiation
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
Show applications key
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.
WB: 1/2000 - 1/5000. Predicted molecular weight: 24 kDa.
Target
Function
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.
Tissue specificity
Expressed in the adult and fetal brain and kidney.
Pathway
Protein modification; protein ubiquitination.
Involvement in disease
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.
Domain
The Elongin BC complex binding domain is also known as BC-box with the consensus [APST]-L-x(3)-C-x(3)-[AILV].
Cellular localization
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.
Target information above from: UniProt accessionP40337
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- pVHL antibody
- G7 protein antibody
- Elongin binding protein antibody
see all
Database links
- Entrez Gene: 7428 Human
- Omim: 193300 Human
- Omim: 608537 Human
- SwissProt: P40337 Human
- Unigene: 517792 Human
- Unigene: 607789 Human
References for Anti-Von Hippel Lindau antibody (ab28434)
This product has been referenced in:
- André H & Pereira TS Identification of an alternative mechanism of degradation of the hypoxia-inducible factor-1alpha. J Biol Chem 283:29375-84 (2008). WB; Human.Read more (PubMed: 18694926) »
See 1 publication for this product
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"