Background:

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). VHL protein has been shown to exist as three distinct isoforms resulting from alternatively spliced transcript variants. 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. Under normoxic conditions, VHL directs the ubiquitylation and subsequent proteosomal degradation of the hypoxia inducible factor 1α (HIF-1α), maintaining very low levels of HIF-1α in the cell. Cellular exposure to hypoxic conditions, or loss of VHL protein function, results in increased HIF-1α 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. In addition to HIF-1α, 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). 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.

Specificity:

Recognizes endogenous levels of Von Hippel Lindau protein.

Alternative names:

Von Hippel-Lindau disease tumor suppressor; Protein G7; pVHL

Species reactivity:

Human

Host:

Mouse

Ig type:

Mouse IgG3. Liquid in PBS containing 50% glycerol, 0.2% BSA and 0.01% sodium azide.

Immunogen:

KLH-conjugated synthetic peptide encompassing a sequence within human Von Hippel Lindau. The exact sequence is proprietary.

Purification & Purity:

The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Molecular weight:

~ 24 kDa

Applications:

IHC

Usage:

IHC (1/100 - 1/300)

Storage:

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Precautions:

For research use only, not for use in diagnostic procedure.

More info:

Email: info@sobekbio.com

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