Rabbit anti-Human VHL Polyclonal Antibody | anti-VHL antibody

VHL, CT (Von Hippel-Lindau Disease Tumor Suppressor, pVHL, Protein G7) (FITC)

Cat. Num. AAA6505875

• Gene Names: VHL; RCA1; VHL1; pVHL; HRCA1
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: VHL; Polyclonal Antibody; CT (Von Hippel-Lindau Disease Tumor Suppressor; pVHL; Protein G7) (FITC); anti-VHL antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human VHL.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-VHL antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:50-1:100; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the C-terminal region of human VHL (KLH).
• Conjugate: FITC
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer.

Western Blot (WB)

(Western blot analysis of VHL antibody (C-term) in HepG2 cell line lysates (35ug/lane). VHL (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(VHL Antibody (C-term) (RB18668) IHC analysis in formalin fixed and paraffin embedded mouse brain tissue followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the VHL Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.)

Flow Cytometry (FC/FACS)

(VHL Antibody (C-term) flow cytometric analysis of HepG2 cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Product Categories/Family for anti-VHL antibody

Antibodies; Disease Markers

References

1. Olmos, G., Cell. Mol. Life Sci. 66(13), 2167-2180 (2009). 2. Hatzimichael, E., Clin Lymphoma Myeloma 9(3), 239-242 (2009). 3. Luu, V.D., Clin. Cancer Res. 15(10), 3297-3304 (2009).

NCBI and Uniprot Product Information

• NCBI GI #: 4507891
• NCBI GeneID: 7428
• NCBI Accession #: NP_000542
• NCBI GenBank Nucleotide #: NM_000551.3
• UniProt Accession #: P40337; Q13599; Q6PDA9; B2RE45
• Molecular Weight: 18,532 Da
• NCBI Official Full Name: von Hippel-Lindau disease tumor suppressor isoform 1
• NCBI Official Synonym Full Names: von Hippel-Lindau tumor suppressor
• NCBI Official Symbol: VHL
• NCBI Official Synonym Symbols: RCA1; VHL1; pVHL; HRCA1
• NCBI Protein Information: von Hippel-Lindau disease tumor suppressor
• UniProt Protein Name: Von Hippel-Lindau disease tumor suppressor
• Protein Family: Von Hippel-Lindau disease tumor suppressor
• UniProt Gene Name: VHL
• UniProt Entry Name: VHL_HUMAN

NCBI Description

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]

Uniprot Description

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 adult 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