| 货号 | 68547S |
| 反应种属 | Human |
| 来源宿主 | Rabbit |
| 应用 | WB |
| 目标/特异性 | VHL Antibody recognizes endogenous levels of total VHL protein. |
| 使用方法 | W |
| 供应商 | CST |
| 灵敏度 | Endogenous |
| 背景 | The 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) (1,2,3). 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 proteosomal 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 lambda (aPKC), and two subunits of the multiprotein RNA Polymerase II complex (RPB1 and RPB7) (5,6,7,8). 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. |
| 存放说明 | -20C |
| 计算分子量 | 18-22 |
| 参考文献 | 1 . Kibel, A. et al. (1995) Science 269, 1444-6. 2 . Pause, A. et al. (1997) Proc Natl Acad Sci U S A 94, 2156-61. 3 . Kamura, T. et al. (2000) Proc Natl Acad Sci U S A 97, 10430-5. 4 . Czyzyk-Krzeska, M.F. and Meller, J. (2004) Trends Mol Med 10, 146-9. 5 . Corn, P.G. et al. (2003) Nat Genet 35, 229-37. 6 . Na, X. et al. (2003) EMBO J 22, 4249-59. 7 . Kuznetsova, A.V. et al. (2003) Proc Natl Acad Sci U S A 100, 2706-11. 8 . Li, Z. et al. (2003) EMBO J 22, 1857-67. |
Western blot analysis of extracts from various cell lines using VHL Antibody (upper) and β-Actin (D6A8) Rabbit mAb #8457 (lower). Note that 786-O is a VHL-null cell line, demonstrating specificity of the antibody. |
