Von Hippel-Lindau Disease Tumor Suppressor (VHL) Antibody

Catalogue No: abx000699
Price: £120.00
(Size: 20 µl)
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VHL Antibody is a Rabbit Polyclonal antibody against VHL. The Von Hippel-Lindau (VHL) protein is a substrate recognition component of an E3 ubiquitin ligase complex containing elongin BC (TCEB1 and TCEB2), cullin 1 (CUL1), and RING-box protein 1 (RBX1) (1,2,3). VHL protein has been shown to exist as three distinct isoforms resulting from alternatively spliced transcript variants (4). Loss of VHL protein function results in a dominantly inherited familial cancer syndrome that manifests as angiomas of the retina, hemangioblastomas of the central nervous system, renal clear-cell carcinomas and pheochromocytomas (4). Under normoxic conditions, VHL directs the ubiquitylation and subsequent proteosomal degradation of the hypoxia inducible factor HIF alpha, maintaining very low levels of HIF alpha in the cell. Cellular exposure to hypoxic conditions, or loss of VHL protein function, results in increased HIF alpha protein levels and increased expression of HIF-induced gene products, many of which are angiogenesis factors such as vascular endothelial growth factor (VEGF). Thus, loss of VHL protein function is believed to contribute to the formation of highly vascular neoplasias (4). In addition to HIF alpha, VHL is known to regulate the ubiquitylation of several other proteins, including tat-binding protein 1 (TBP-1), the atypical protein kinase C lambda (aPKC), and two subunits of the multiprotein RNA Polymerase II complex (RPB1 and RPB7) (5,6,7,8). Interactions with elongin BC, RPB1, RPB7 and the pVHL-associated KRAB-A domain containing protein (VHLaK) suggest that VHL may also play a more direct role in transcriptional repression.

Target VHL
Clonality Polyclonal
Reactivity Human, Mouse, Rat
Tested Applications WB, IHC, IF/ICC
Host Rabbit
Recommended dilutions WB: 1/500 - 1/2000, IHC: 1/50 - 1/200, IF/ICC: 1/50 - 1/200. Optimal dilutions/concentrations should be determined by the end user.
Conjugation Unconjugated
Immunogen Recombinant protein of human VHL.
Isotype IgG
Form Liquid
Purification Purified by affinity chromatography.
Storage Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
UniProt Primary AC P40337 (UniProt, ExPASy)
UniProt Secondary AC B2RE45, Q13599, Q6PDA9
UniProt Entry Name VHL_HUMAN
Gene Symbol VHL
GeneID 7428
KEGG hsa:7428
String 9606.ENSP00000256474
Molecular Weight Calculated MW: 18 kDa, 19 kDa, 24 kDa
Observed MW: 23 kDa
Buffer PBS, pH 7.3, 0.02% sodium azide, 50% glycerol.
Concentration > 0.5 mg/ml
Availability Shipped within 5-10 working days.
Note This product is for research use only.
Research Articles on VHL

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