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VHL / Von Hippel Lindau

von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase

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.

Gene Name: von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase
Synonyms: VHL, Elongin binding protein, HRCA1, RCA1, VHL1, Von Hippel-Lindau syndrome, Protein G7, PVHL
Target Sequences: NM_000551 NP_000542.1 P40337

Publications (2)

1
Regulation of E-cadherin expression by VHL and hypoxia-inducible factor. Esteban MA, Tran MG, Harten SK, Hill P, Castellanos MC, Chandra A, Raval R, O'brien TS, Maxwell PH. Cancer research. 2006 66:3567-75. [PubMed:16585181]
2
MCM proteins are negative regulators of hypoxia-inducible factor 1. Hubbi ME, Luo W, Baek JH, Semenza GL. Molecular cell. 2011 42:700-12. (WB, IP; Human) [PubMed:21658608] [PMC:PMC3131976]

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Products
Proteins (10)
Over-Expression Lysate (4)
Recombinant (6)
VHL / Von Hippel Lindau (10)
No (10)
6His, N-terminus (1)
GST, N-terminus (1)
His (1)
His, N-Terminal (1)
His, N-terminus (1)
Myc-DDK (Flag) (5)
Human (7)
Rat (1)
293T Cells (2)
E. coli (4)
HEK 293 Cells (3)
Wheat Germ Extract (1)
Purified (2)
VHL / Von Hippel Lindau Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
19.5 kDa
20 µg/$150
VHL / Von Hippel Lindau Protein
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E. coli
His
100 µg/$317; 500 µg/$526; 20 µg/$245
VHL / Von Hippel Lindau Protein - 12.5% SDS-PAGE of human VHL stained with Coomassie Blue
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Wheat Germ Extract
GST, N-terminus
46.1 kDa
2 µg/$439
VHL / Von Hippel Lindau Protein - (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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E. coli
6His, N-terminus
28.2 kDa
1 mg/$1,679; 100 µg/$500; 20 µg/$364
VHL / Von Hippel Lindau Protein - Recombinant  Von Hippel Lindau Tumor Suppressor By SDS-PAGE
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E. coli
His, N-terminus
23.3 kDa
10 µg/$284; 50 µg/$462; 200 µg/$922; 1 mg/$2,042; 5 mg/$3,221; 2 mg/$2,231
VHL / Von Hippel Lindau Protein - Purified recombinant protein VHL was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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HEK 293 Cells
Myc-DDK (Flag)
24 kDa
20 µg/$1,107
VHL / Von Hippel Lindau Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
24 kDa
100 µg/$494
VHL / Von Hippel Lindau Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
24 kDa
20 µg/$150
VHL / Von Hippel Lindau Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
HEK 293 Cells
Myc-DDK (Flag)
19.5 kDa
100 µg/$494
VHL / Von Hippel Lindau Protein
Select
E. coli
His, N-Terminal
24 kDa
50 µg/$419
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For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).