pVHL, Monoclonal Antibody

Anti-pVHL [ABT-PVHL]; Mouse monoclonal [ABT-PVHL] antibody to PVHL; G7 protein; pVHL; Von Hippel-Lindau disease tumor suppressor; von Hippel-Lindau syndrome gene

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

Monoclonal

IgG

ABT-PVHL

Mouse

Immunogen Affinity Purified

0.5% Bovine Serum Albumin (BSA), 0.1% Kathon CG/ICP, 10x Tris-Buffered Saline (TBS), Type 3 MilliQ H2O and 50% Glycerol.

1mg/ml (lot specific)

Store at -20 degree C for 1 year.

Small volumes of anti-pVHL 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.

Monoclonal IHC-Gold Antibody

Immunohistochemistry (IHC)

Dilution: 1:50-200

von Hippel-Lindau tumor suppressor

von Hippel-Lindau disease tumor suppressor

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; mitochondrion; membrane; endoplasmic reticulum; nucleus; cytosol

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

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

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

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