产品简要
公司名称 :
MyBioSource
产品类型 :
抗体
产品名称 :
VHL多克隆抗体
目录 :
MBS125962
规格 :
0.05毫升
价格 :
230美元
克隆性 :
多克隆
宿主 :
共轭标签 :
未共轭
反应物种 :
人类, 小鼠, 大鼠
应用 :
免疫印迹, 免疫组化, 免疫细胞化学, 免疫印迹基因敲除验证
更多信息或购买 :
文章摘录数: 1
出版应用/物种/样本/稀释参考文献
  • 免疫印迹基因敲除验证; 人类; 图 1
German P, Bai S, Liu X, Sun M, Zhou L, Kalra S, et al. Phosphorylation-dependent cleavage regulates von Hippel Lindau proteostasis and function. Oncogene. 2016;35:4973-80 pubmed 出版商
产品信息
目录号 :
MBS125962
产品类型 :
抗体
产品全称 :
VHL多克隆抗体
产品简称 :
VHL
产品名称同义词 :
Von Hippel-Lindau disease tumor suppressor; pVHL; Protein G7; VHL
其他名称 :
Von Hippel-Lindau disease tumor suppressor; Von Hippel-Lindau disease tumor suppressor; von Hippel-Lindau disease tumor suppressor; protein G7; elongin binding protein; von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase; Protein G7; pVHL
产品基因名称 :
VHL
其他基因名称 :
VHL;VHL;RCA1;VHL1;pVHL;HRCA1
UniProt数据库进入名 :
VHL_HUMAN
克隆性 :
多克隆
抗体亚型 :
IgG
宿主 :
反应物种 :
人类, 小鼠, 大鼠
序列长度 :
213
纯度 :
亲和纯化
储存稳定性 :
Store at -20 degree C. Avoid freeze/thaw cycles
检测过的应用 :
免疫印迹(免疫印迹), 免疫组化(免疫组化), 免疫荧光(IF)
应用笔记 :
WB: 1:500 - 1:2000。 IHC: 1:50 - 1:200。 IF: 1:20 - 1:100
其它信息1 :
Species: Human. Category: Primary Antibody
其他信息2 :
Immunogen: Recombinant protein of human VHL. Calculated Molecular Weight: 24kDa. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
产品种类 :
多克隆
产品描述 :
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 HIF alpha, maintaining very low levels of HIF alpha in the cell. Cellular exposure to hypoxic conditions, or loss of VHL protein function, results in increased HIF alpha 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 alpha, 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.
NCBI GI登录号 :
4033778
NCBI登录号 :
P40337.2
UniProt数据库登录号 :
P40337
NCBI信号通路 :
Adaptive Immune System Pathway (366160); Antigen Processing: Ubiquitination Proteasome Degradation Pathway (366162); Cellular Response To Hypoxia Pathway (645259); Cellular Responses To Stress Pathway (645258); Class I MHC Mediated Antigen Processing Presentation Pathway (366161); ECV Complex Pathway (413450); ECV Complex Pathway (890606); HIF-1 Signaling Pathway (695200); HIF-2-alpha Transcription Factor Network Pathway (137956); Hypoxic And Oxygen Homeostasis Regulation Of HIF-1-alpha Pathway (138056)
NCBI总结 :
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]
UniProt数据库总结 :
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: Ubiquitin ligase; Tumor suppressor. 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
尺寸1 :
0.05毫升
价格1 :
230美元
尺寸2 :
0.1毫升
价格2 :
305
size3 :
0.2毫升
价格3 :
460
更多信息或购买 :
公司信息
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com
1-858-633-0165
公司总部: 美国
MyBioSource,LLC最初由三名热情澎湃的提供高品质试剂的科学家联合创立于温哥华,公司愿景是“生物研究试剂的源头”,现在位于圣地亚哥市。