{"product_id":"cst-68547s","title":"CST,  68547S, VHL Antibody","description":"Polyclonal Antibody for studying VHL. Validated for Western Blotting,Simple Western™. Highly specific and rigorously validated in-house, VHL Antibody (CST #68547) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nWestern Blotting: 1:1000\nSimple Western™: 1:10 - 1:50\n\u003cb\u003eStorage\u003c\/b\u003e\nSupplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg\/ml BSA and 50% glycerol. Store at -20°C. Do not aliquot the antibody.\n\u003cb\u003eProtocol\u003c\/b\u003e\nAvailable protocols: Western Blotting\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nVHL Antibody recognizes endogenous levels of total VHL protein.\nSpecies Reactivity: Human\n\u003cb\u003eSource \/ Purification\u003c\/b\u003e\nPolyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues near the carboxy terminus of human VHL protein.\n\u003cb\u003eBackground\u003c\/b\u003e\nThe von Hippel-Lindau (VHL) protein is a substrate recognition component of an E3 ubiquitin ligase complex containing elongin BC (TCEB1 and TCEB2), cullin 1 (CUL1), and RING-box protein 1 (RBX1) (1-3). VHL protein has been shown to exist as three distinct isoforms resulting from alternatively spliced transcript variants (4). Loss of VHL protein function results in a dominantly inherited familial cancer syndrome that manifests as angiomas of the retina, hemangioblastomas of the central nervous system, renal clear cell carcinomas, and pheochromocytomas (4). Under normoxic conditions, VHL directs the ubiquitylation and subsequent proteasomal degradation of the hypoxia-inducible factor 1α (HIF-1α), maintaining very low levels of HIF-1α in the cell. Cellular exposure to hypoxic conditions, or loss of VHL protein function, results in increased HIF-1α protein levels and increased expression of HIF-induced gene products, many of which are angiogenesis factors such as vascular endothelial growth factor (VEGF). Thus, loss of VHL protein function is believed to contribute to the formation of highly vascular neoplasias (4). In addition to HIF-1α, VHL is known to regulate the ubiquitylation of several other proteins, including tat-binding protein-1 (TBP-1), the atypical protein kinase C (aPKC) lambda, and two subunits of the multiprotein RNA polymerase II complex (RPB1 and RPB7) (5-8). Interactions with elongin BC, RPB1, RPB7, and the pVHL-associated KRAB-A domain-containing protein (VHLaK) suggest that VHL may also play a more direct role in transcriptional repression.\n\u003cb\u003eAlternate Names\u003c\/b\u003e\nelongin binding protein; HRCA1; Protein G7; pVHL; RCA1; VHL; VHL1; von Hippel-Lindau disease tumor suppressor; von Hippel-Lindau tumor suppressor; von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase\n\n\u003cb\u003eSpecification\u003c\/b\u003e\n\nREACTIVITY: H\nSENSITIVITY: Endogenous\nMW (kDa): 18-22\nSOURCE: Rabbit","brand":"CST","offers":[{"title":"Default Title","offer_id":46799855976617,"sku":"68547S","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-68547s","provider":"Iright","version":"1.0","type":"link"}