货号 |
bs-1367R-1 |
品牌 |
|
浓度 |
|
货期 |
现货 |
英文名称 |
Rabbit Anti-VHL antibody |
中文名称 |
Rabbit Anti-VHL antibody |
研究领域 |
肿瘤,细胞生物,免疫学,染色质和核信号,转录调节因子,新陈代谢,细胞分化,表观遗传学,泛素, |
英文别名 |
Von Hippel Lindau; von Hippel-Lindau syndrome protein homolog; Hippel-Lindau disease tumor suppressor VHL; von Hippel-Lindau tumor suppressor isoform 1; VHL; HRCA1; RCA1; VHL1; von Hippel-Lindau disease tumor suppressor isoform 2;
pVHL; pVHL; G7 protein; |
反应物种(已验证) |
Human,Rat |
反应物种(预测) |
Mouse,Dog,Cow |
产品应用(已验证) |
IHC,FCM |
产品应用(可推荐) |
IF,ELISA |
推荐稀释比例 |
Elisa=1:5000-10000,IHC-P=1:100-500,IHC-F=1:100-500,Flow Cyt=1ug/test,IF=1:100-500, |
克隆类型 |
多克隆 |
抗体来源 |
Rabbit |
理论分子量 |
24 |
细胞定位 |
细胞核,细胞浆,细胞膜 |
性状 |
Liquid |
免疫原 |
KLH conjugated synthetic peptide derived from human VHL |
抗原表位 |
101-213/213 |
亚型 |
IgG |
纯化方法 |
affinity purified by Protein A |
SUBCELLULAR |
Isoform 1: Cytoplasm. Membrane; Peripheral membrane protein. Nucleus. Note=Found predominantly in the cytoplasm and with less amounts nuclear or membrane-associated. Colocalizes with ADRB2 at the cell membrane. Isoform 3: Cytoplasm. Nucleus. Note=Equal |
Tissue |
Expressed in the adult and fetal brain and kidney. |
SUBUNIT |
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 compl |
Function |
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) |
DISEASE |
Defects in VHL are a cause of susceptibility to pheochromocytoma (PCC) [MIM:171300]. A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinep |
SWISS |
P40337 |
Gene ID |
7428 |
保存条件 |
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note |
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 |
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]. |