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VHL recombinant protein

Scan QR to view Datasheet Catalog #    MBS203102
SDS-PAGE
Unit / Price
0.1 mg  /  $255 +1 FREE 8GB USB
0.5 mg  /  $535 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

VHL, Recombinant Protein

★Popular Item★
 Also Known As   

VHL, 1-154aa; Human, His-tagged, Recombinant, E.coli

 Product Synonym Names    Von Hippel-Lindau tumor suppressor isoform 1; HRCA1; RCA1; VHL1
 Product Gene Name   

VHL recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence    MGSSHHHHHH SSGLVPRGSH MPRRAENWDE AEVGAEEAGV EEYGPEEDGG EESGAEESGP EESGPEELGA EEEMEAGRPR PVLRSVNSRE PSQVIFCNRS PRVVLPVWLN FDGEPQPYPT LPPGTGRRIH SYRGHLWLFR DAGTHDGLLV NQTELFVPSL NVDGQPIFAN ITLP
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 Host    E.coli
 Species Reactivity    Human
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 Purity/Purification    > 95% by SDS-PAGE
 Form/Format    Liquid. In phosphate-buffered Saline(pH 7.4), containing 1mM DTT, 2mM EDTA
 Concentration    1 mg/ml (determined by Bradford assay) (lot specific)
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 Preparation and Storage    Can be stored at 4 degree C short term (1-2 weeks). For long term storage, aliquot and store at -20 degree C or -70 degree C. Avoid repeated freezing and thawing cycles.
 Other Notes    Small volumes of VHL recombinant protein 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.
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Product Description specifically for VHL recombinant protein

   Von Hippel-Lindau disease(VHL) is a dominant inherited syndrome characterized by the predisposition to develop various kinds of benign and malignant tumors, including clear cell renal carcinomas, pheochromocytomas and hemangioblastomas of the central nervous system and retina. VHL syndrome is caused by germline mutation in the VHL tumor suppressor, and VHL tumors are associated with loss or mutation of the remaining wild-type allele. VHL has two domains: a roughly 100-residue NH2-terminal domain rich in beta sheet(beta-domain) and a smaller alpha-helical domain(alpha-domain), held together by two linkers and a polar interface. VHL protein is also involved in the degradation of hypoxia-inducible factor (HIF). VHL beta-domain(1-154aa) was overexpressed in E.coli and purified by using conventional chromatography techniques.
 Product Categories/Family for VHL recombinant protein    Cancer
 Applications Tested/Suitable for VHL recombinant protein   

SDS-PAGE

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 SDS-PAGE of VHL recombinant protein    VHL recombinant protein SDS-PAGE image
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NCBI/Uniprot data below describe general gene information for VHL. It may not necessarily be applicable to this product.
 NCBI GI #    4507891
 NCBI GeneID    7428
 NCBI Accession #    NP_000542 [Other Products]
 NCBI GenBank Nucleotide #    NM_000551.3 [Other Products]
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 UniProt Secondary Accession #    Q13599; Q6PDA9; B2RE45 [Other Products]
 UniProt Related Accession #    P40337 [Other Products]
 Molecular Weight    19.2 kDa (174 aa), confirmed by MALDI-TOF.
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 NCBI Official Full Name    von Hippel-Lindau disease tumor suppressor isoform 1
 NCBI Official Synonym Full Names    von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase
 NCBI Official Symbol    VHL [Similar Products]
 NCBI Official Synonym Symbols   
RCA1; VHL1; pVHL; HRCA1
[Similar Products]
 NCBI Protein Information    von Hippel-Lindau disease tumor suppressor; elongin binding protein; protein G7
 UniProt Protein Name    Von Hippel-Lindau disease tumor suppressor
 UniProt Synonym Protein Names   
Protein G7; pVHL
 Protein Family    Von Hippel-Lindau disease tumor suppressor
 UniProt Gene Name    VHL [Similar Products]
 UniProt Entry Name    VHL_HUMAN
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 NCBI Summary for VHL    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]
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 UniProt Comments for VHL    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: Tumor suppressor; Ubiquitin ligase

Chromosomal Location of Human Ortholog: 3p25.3

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

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; 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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Product References and Citations for VHL recombinant protein

   Latif F., et al. (1993) Science. 260(5112):1317-20
Duan DR., et al. (1995) PNAS. 92(14):6459-63
Maxwell PH., et al. (1999) Nature 399(6733):271-5
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 Research Articles on VHL    1. Exon 1 methylation may be an alternate mechanism of VHL gene silencing in renal cell carcinoma in addition to mutation and promoter methylation.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with VHL recombinant proteinPathways associated with VHL recombinant protein
 Reference Product  PubMed Publications
 HIF1A recombinant protein  >528 publications with VHL and HIF1A
 CUL2 recombinant protein  >56 publications with VHL and CUL2
 EGLN1 recombinant protein  >45 publications with VHL and EGLN1
 RBX1 recombinant protein  >29 publications with VHL and RBX1
 EGLN3 recombinant protein  >26 publications with VHL and EGLN3
 EPAS1 recombinant protein  >14 publications with VHL and EPAS1
 TCEB1 recombinant protein  >5 publications with VHL and TCEB1
 UBC recombinant protein  >2 publications with VHL and UBC
 TCEB2 recombinant protein  >2 publications with VHL and TCEB2
 Products by Pathway  Pathway Diagram
 Adaptive Immune System Pathway antibodies  Adaptive Immune System Pathway Diagram
 Antigen Processing: Ubiquitination & Proteasome Degradation Pathway antibodies  Antigen Processing: Ubiquitination & Proteasome Degradation Pathway Diagram
 Cellular Response To Hypoxia Pathway antibodies  Cellular Response To Hypoxia Pathway Diagram
 Cellular Responses To Stress Pathway antibodies  Cellular Responses To Stress Pathway Diagram
 Class I MHC Mediated Antigen Processing & Presentation Pathway antibodies  Class I MHC Mediated Antigen Processing & Presentation Pathway Diagram
 ECV Complex Pathway antibodies  ECV Complex Pathway Diagram
 ECV Complex Pathway antibodies  ECV Complex Pathway Diagram
 HIF-1 Signaling Pathway antibodies  HIF-1 Signaling Pathway Diagram
 HIF-2-alpha Transcription Factor Network Pathway antibodies  HIF-2-alpha Transcription Factor Network Pathway Diagram
 Hypoxic And Oxygen Homeostasis Regulation Of HIF-1-alpha Pathway antibodies  Hypoxic And Oxygen Homeostasis Regulation Of HIF-1-alpha Pathway Diagram
Diseases associated with VHL recombinant proteinOrgans/Tissues associated with VHL recombinant protein
 Disease Name  Pubmed Publications
 von Hippel-Lindau Disease Antibodies  >1613 publications with VHL and von Hippel-Lindau Disease
 Carcinoma Antibodies  >1398 publications with VHL and Carcinoma
 Carcinoma, Renal Cell Antibodies  >1204 publications with VHL and Carcinoma, Renal Cell
 Kidney Diseases Antibodies  >1067 publications with VHL and Kidney Diseases
 Kidney Neoplasms Antibodies  >1065 publications with VHL and Kidney Neoplasms
 Cardiovascular Diseases Antibodies  >1054 publications with VHL and Cardiovascular Diseases
 Pheochromocytoma Antibodies  >496 publications with VHL and Pheochromocytoma
 Brain Diseases Antibodies  >196 publications with VHL and Brain Diseases
 Hypertension Antibodies  >104 publications with VHL and Hypertension
 Adenoma Antibodies  >104 publications with VHL and Adenoma
 Organ/Tissue Name  Pubmed Publications
 Kidney Antibodies  >1142 publications with VHL and Kidney
 Vascular Antibodies  >545 publications with VHL and Vascular
 Blood Antibodies  >372 publications with VHL and Blood
 Brain Antibodies  >170 publications with VHL and Brain
 Pancreas Antibodies  >135 publications with VHL and Pancreas
 Liver Antibodies  >107 publications with VHL and Liver
 Eye Antibodies  >106 publications with VHL and Eye
 Lung Antibodies  >101 publications with VHL and Lung
 Bone Antibodies  >95 publications with VHL and Bone
 Ear Antibodies  >59 publications with VHL and Ear
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