VHL / Von Hippel Lindau, Monoclonal Antibody

Anti-VHL / Von Hippel Lindau Antibody (clone 52A11) IHC-plus; VHL; Elongin binding protein; HRCA1; RCA1; VHL1; Von Hippel-Lindau syndrome; Protein G7; PVHL; Human VHL; Von Hippel Lindau

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG2b,k

52A11

Mouse

Protein G Purified

PBS, pH 7.4, 0.1% sodium azide. Sourced in Ascites.

1 mg/ml (lot specific)

Long term: -20 degree C; Short term: +4 degree C; Avoid freeze-thaw cycles.

Small volumes of anti-VHL antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)

ELISA, IHC-P (15 ug/ml), WB (1:500 - 1:1000)
Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary anti...

Anti-VHL antibody IHC of human pancreas. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 15 ug/ml.

HepG2 cell lysate was resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human VHL antibody (1:500). Protein was visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system.

18,532 Da

von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase

von Hippel-Lindau disease tumor suppressor; protein G7; elongin binding protein

Von Hippel-Lindau disease tumor suppressor

VHL_HUMAN

Von Hippel-Lindau syndrome (VHL) is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign tumors. A germline mutation of this gene is the basis of familial inheritance of VHL syndrome. The protein encoded by this gene is a component of the protein complex that includes elongin B, elongin C, and cullin-2, and possesses ubiquitin ligase E3 activity. This protein is involved in the ubiquitination and degradation of hypoxia-inducible-factor (HIF), which is a transcription factor that plays a central role in the regulation of gene expression by oxygen. RNA polymerase II subunit POLR2G/RPB7 is also reported to be a target of this protein. Alternatively spliced transcript variants encoding distinct isoforms have been observed. [provided by RefSeq, Jul 2008]

VHL: 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. Ubiquitinates, in an oxygen-responsive manner, ADRB2. Component of the VCB (VHL-Elongin BC-CUL2) complex; this complex acts as a ubiquitin-ligase E3 and directs proteasome- dependent degradation of targeted proteins. Interacts with CUL2; this interaction is dependent on the integrity of the trimeric VBC complex. Interacts (via the beta domain) with HIF1A (via the NTAD domain); this interaction mediates degradation of HIF1A in normoxia and, in hypoxia, prevents ubiqitination and degradation of HIF1A by mediating hypoxia-induced translocation to the nucleus, a process which requires a hypoxia-dependent regulatory signal. Interacts with ADRB2; the interaction, in normoxia, is dependent on hydroxylation of ADRB2 and the subsequent VCB- mediated ubiquitination and degradation of ADRB2. Under hypoxia, hydroxylation, interaction with VHL, ubiquitination and subsequent degradation of ADRB2 are dramatically decreased. Interacts with RNF139, USP33 and PHF17. Found in a complex composed of LIMD1, VHL, EGLN1/PHD2, TCEB2 AND CUL2. Isoform 1 and isoform 3 interact with LIMD1 (via LIM zinc-binding 2), AJUBA (via LIM domains) and WTIP (via LIM domains). Interacts with EPAS1. Expressed in the ***** and fetal brain and kidney. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Ubiquitin ligase; Tumor suppressor

Chromosomal Location of Human Ortholog: 3p25.3

Cellular Component: nucleoplasm; intermediate filament cytoskeleton; membrane; mitochondrion; endoplasmic reticulum; nucleus; cytosol

Molecular Function: protein binding; enzyme binding; ubiquitin-protein ligase activity; transcription factor binding

Biological Process: negative regulation of cell proliferation; regulation of transcription, DNA-dependent; protein stabilization; positive regulation of transcription, DNA-dependent; cell morphogenesis; protein ubiquitination; negative regulation of transcription from RNA polymerase II promoter; positive regulation of cell differentiation; proteolysis; negative regulation of apoptosis

Disease: Erythrocytosis, Familial, 2; Von Hippel-lindau Syndrome; Renal Cell Carcinoma, Nonpapillary; Pheochromocytoma

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