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

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

Cat. Num. AAA649554

• Gene Names: VHL; RCA1; VHL1; pVHL; HRCA1
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Affinity Purified; Purified by Protein G affinity chromatography.
• Synonyms: VHL; Polyclonal Antibody; NT (Von Hippel-Lindau Disease Tumor Suppressor; pVHL; Protein G7); Anti -VHL; anti-VHL antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human VHL.
• Purity/Purification: Affinity Purified; Purified by Protein G affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, 0.09% sodium azide.

• Applicable Applications for anti-VHL antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in ELISA and Western Blot.; Dilution: ELISA: 1:1,000; Western Blot: 1:50-1:100
• Immunogen: Synthetic peptide selected from the N-terminal region of human VHL (KLH).
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in HepG2 cell line lysates (35ug) using MBS649554. VHL (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis in formalin fixed and paraffin embedded mouse brain tissue using MBS649554 followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of MBS649554 for immunohistochemistry.)

Immunofluorescence (IF)

(Confocal immunofluorescent analysis with HepG2 cell using MBS649554 followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). Actin filaments have been labeled with Alexa Fluor 555 phalloidin (red). DAPI was used to stain the cell nuclear (blue).)

Flow Cytometry (FC/FACS)

(Flow Cytometric analysis of HepG2 cells using MBS649554 (right histogram) compared to a negative control cell (left histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Related Product Information for anti-VHL antibody

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 VHL gene is the basis of familial inheritance of VHL syndrome. The protein 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.

Product Categories/Family for anti-VHL antibody

Antibodies; Abs to Disease Markers

NCBI and Uniprot Product Information

• NCBI GI #: 38045906
• NCBI GeneID: 7428
• NCBI Accession #: NP_937799.1
• NCBI GenBank Nucleotide #: NM_198156.2
• UniProt Accession #: P40337; Q13599; Q6PDA9; B2RE45
• Molecular Weight: 24,153 Da
• NCBI Official Full Name: von Hippel-Lindau disease tumor suppressor isoform 2
• NCBI Official Synonym Full Names: von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase
• NCBI Official Symbol: VHL
• NCBI Official Synonym Symbols: RCA1; VHL1; pVHL; HRCA1
• NCBI Protein Information: von Hippel-Lindau disease tumor suppressor; protein G7; elongin binding protein
• 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

Function: 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. Ref.11 Ref.13 Ref.20

Pathway: Protein modification; protein ubiquitination.

Subunit structure: 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. Ref.8 Ref.10 Ref.12 Ref.13 Ref.14 Ref.15 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21

Subcellular location: Isoform 1: Cytoplasm. Membrane; Peripheral membrane protein. Nucleus. Note: Found predominantly in the cytoplasm and with less amounts nuclear or membrane-associated. Colocalizes with ADRB2 at the cell membrane. Ref.11 Ref.20Isoform 3: Cytoplasm. Nucleus. Note: Equally distributed between the nucleus and the cytoplasm but not membrane-associated. Ref.11 Ref.20

Tissue specificity: Expressed in the adult and fetal brain and kidney.

Developmental stage: At 4-10 weeks pc, strong expression in the developing central nervous system, kidneys, testis and lung. Differentially expressed within renal tubules. Ref.7

Domain: The Elongin BC complex binding domain is also known as BC-box with the consensus [APST]-L-x(3)-C-x(3)-[AILV].

Involvement in disease: Pheochromocytoma (PCC) [MIM:171300]: A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry.von Hippel-Lindau disease (VHLD) [MIM:193300]: VHLD is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign neoplasms, most frequently retinal, cerebellar and spinal hemangioblastoma, renal cell carcinoma (RCC), pheochromocytoma, and pancreatic tumors. VHL type 1 is without pheochromocytoma, type 2 is with pheochromocytoma. VHL type 2 is further subdivided into types 2A (pheochromocytoma, retinal angioma, and hemangioblastomas without renal cell carcinoma and pancreatic cyst) and 2B (pheochromocytoma, retinal angioma, and hemangioblastomas with renal cell carcinoma and pancreatic cyst).Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.1 Ref.12 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32 Ref.33 Ref.34 Ref.35 Ref.36 Ref.37 Ref.38 Ref.40 Ref.41 Ref.42 Ref.49Familial erythrocytosis 2 (ECYT2) [MIM:263400]: An autosomal recessive disorder characterized by an increase in serum red blood cell mass, hypersensitivity of erythroid progenitors to erythropoietin, increased erythropoietin serum levels, and normal oxygen affinity. Patients with ECYT2 carry a high risk for peripheral thrombosis and cerebrovascular events.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.45 Ref.48Renal cell carcinoma (RCC) [MIM:144700]: Renal cell carcinoma is a heterogeneous group of sporadic or hereditary carcinoma derived from cells of the proximal renal tubular epithelium. It is subclassified into clear cell renal carcinoma (non-papillary carcinoma), papillary renal cell carcinoma, chromophobe renal cell carcinoma, collecting duct carcinoma with medullary carcinoma of the kidney, and unclassified renal cell carcinoma. Clear cell renal cell carcinoma is the most common subtype.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.43