VHL (von Hippel-Lindau tumor suppressor protein)

Catalog #: RP-106

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Product Name	VHL (von Hippel-Lindau tumor suppressor protein)
Size	5,000 U
Description	von Hippel-Lindau (VHL) disease is a hereditary cancer with a predilection for the central nervous system and retina (1). The von Hippel-Lindau tumor suppressor gene is mutated in families with von Hippel-Lindau disease and encodes a protein (VHL) of 213 amino acids with an acidic pentapeptide motif in the N-terminus (2-4). Mutations in the VHL gene result in constitutive expression of many hypoxi-induced genes, at least in part because of increases in the cellular level of hypoxia-inducible transcription factor HIF-1a (5). VHL protein binds to elongin B, elongin C, and Cul2 to form a stable complex that targets hypoxia inducibal factors (HIFs) for degradation and transcriptional regulation (6,7). In addition, VHL protein has also been shown to interact with specific protein kinase C isoforms (8), histone deacetylases and HIF-1 inhibitor (FIH-1) (9).
Applications	in vitro transcriptional activation protein-protein interaction assays
Source	Baculovirus-insect cell.
Fusion Tag	6X His
Purification Method	FPLC
Unit Definition (Activity)	1 unit equals to 1 nanogram of purified protein. 30-100 units (ng) are required for an in vitro transcription assay and protein-protein interaction assay.
Purity	~ 95% as determined by SDS-PAGE
Gene ID	7428
Accession #	NM_000551
Gene Name	von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase
Gene Symbol	VHL
Gene Synonyms	RCA1; VHL1; pVHL; HRCA1
MW	26.0 kDa
Protein Sequence	MPRRAENWDE AEVGAEEAGV EEYGPEEDGG EESGAEESGP EESGPEELGA EEEMEAGRPR PVLRSVNSRE PSQVIFCNRS PRVVLPVWLN FDGEPQPYPT LPPGTGRRIH SYRGHLWLFR DAGTHDGLLV NQTELFVPSL NVDGQPIFAN ITLPVYTLKE RCLQVVRSLV KPENYRRLDI VRSLYEDLED HPNVQKDLER LTQERIAHQR MGD
Storage Buffer	20mM Tris-HCl pH7.9,100mM NaCl, 0.2mM EDTA, 1mM DTT and 20% glycerol
Storage	-20 to -80 °C.
Shipping	4°C or dry ice
References	1. Singh, AD. et al., (2001) Survey of Ophthalmology 46, 117-134 2. Latif, F. et al., (1993) Science 260, 1317-1320 3. Linehan, WM. et al., (1995) J. Am. Med. Assoc. 273, 564-570 4. Duan, DR. et al., (1995) Proc. Natl. Acad. Sci. USA 92, 6459-6463 5. Kamura, T. et al., (2000) Proc. Natl. Acad. Sci. USA 97, 10430-10435 6. Duan, DR. et al., (1995) Science 269, 1402-1406 7. Iwai, K. et al., (1999) Proc. Natl. Acad. Sci. USA 96, 12436-12441 8. Pal, S. et al., (1997) J Biol. Chem. 272, 27509-27512 9. Mahon, PC. et al., (2001) Genes & Dev. 15, 2675-2686

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