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腦視網(wǎng)膜血管瘤G7蛋白抗體（逢希伯-林道氏?。?/dt>
  • 產(chǎn)品貨號(hào)：
    BN41700R
  • 中文名稱：
    腦視網(wǎng)膜血管瘤G7蛋白抗體（逢希伯-林道氏病）
  • 英文名稱：
    Rabbit anti-VHL Polyclonal antibody
  • 品牌：
    Biorigin

  • 貨號(hào)

    產(chǎn)品規(guī)格

    售價(jià)

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  • BN41700R-50ul

    50ul

    ￥1486.00

    交叉反應(yīng)：Human,Rat(predicted:Mouse,Dog,Cow) 推薦應(yīng)用：IHC-P,IHC-F,IF,Flow-Cyt,ELISA

  • BN41700R-100ul

    100ul

    ￥2360.00

    交叉反應(yīng)：Human,Rat(predicted:Mouse,Dog,Cow) 推薦應(yīng)用：IHC-P,IHC-F,IF,Flow-Cyt,ELISA

  • BN41700R-200ul

    200ul

    ￥3490.00

    交叉反應(yīng)：Human,Rat(predicted:Mouse,Dog,Cow) 推薦應(yīng)用：IHC-P,IHC-F,IF,Flow-Cyt,ELISA

產(chǎn)品描述

英文名稱VHL
中文名稱腦視網(wǎng)膜血管瘤G7蛋白抗體（逢希伯-林道氏?。?/td>
別    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; Elongin binding protein; Protein G7; VHL 1; VHL_HUMAN; VHL1; VHLH; Von Hippel Lindau disease tumor suppressor; von Hippel Lindau syndrome; von Hippel Lindau tumor suppressor; Von Hippel-Lindau disease tumor suppressor.   
研究領(lǐng)域腫瘤  細(xì)胞生物  免疫學(xué)  染色質(zhì)和核信號(hào)  轉(zhuǎn)錄調(diào)節(jié)因子  細(xì)胞分化  新陳代謝  表觀遺傳學(xué)  泛素  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Human,  (predicted: Mouse, Rat, Dog, Cow, )
產(chǎn)品應(yīng)用ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1ug/test IF=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量24kDa
細(xì)胞定位細(xì)胞核 細(xì)胞漿 細(xì)胞膜 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human VHL:101-213/213 
亞    型IgG
純化方法affinity purified by Protein A
儲(chǔ) 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹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].

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) under normoxic conditions. Involved in transcriptional repression through interaction with HIF1A, HIF1AN and histone deacetylases. Ubiquitinates, in an oxygen-responsive manner, ADRB2.

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 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.

Subcellular Location:
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=Equally distributed between the nucleus and the cytoplasm but not membrane-associated.

Tissue Specificity:
Expressed in the adult and fetal brain and kidney.

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 epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent.
Defects in VHL are the cause of von Hippel-Lindau disease (VHLD) [MIM:193300]. VHLD is a dominantly inherited familial cancer syndrome characterized by the development of retinal angiomatosis, cerebellar and spinal hemangioblastoma, renal cell carcinoma (RCC), phaeochromocytoma and pancreatic tumors. VHL type 1 is without pheochromocytoma, type 2 is with pheochromocytoma. VHL type 2 is further subdivided into types 2A (pheochromocytoma, retinal angioma, and hemangioblastomas without renal cell carcinoma and pancreatic cyst) and 2B (pheochromocytoma, retinal angioma, and hemangioblastomas with renal cell carcinoma and pancreatic cyst). VHL type 2C refers to patients with isolated pheochromocytoma without hemangioblastoma or renal cell carcinoma. The estimated incidence is 3/100000 births per year and penetrance is 97% by age 60 years.
Defects in VHL are the cause of familial erythrocytosis type 2 (ECYT2) [MIM:263400]; also called VHL-dependent polycythemia or Chuvash type polycythemia. ECYT2 is an autosomal recessive disorder characterized by an increase in serum red blood cell mass, hypersensitivity of erythroid progenitors to erythropoietin, increased erythropoietin serum levels, and normal oxygen affinity. Patients with ECYT2 carry a high risk for peripheral thrombosis and cerebrovascular events.
Defects in VHL are a cause of renal cell carcinoma (RCC) [MIM:144700]. Renal cell carcinoma is a heterogeneous group of sporadic or hereditary carcinoma derived from cells of the proximal renal tubular epithelium. It is subclassified into clear cell renal carcinoma (non-papillary carcinoma), papillary renal cell carcinoma, chromophobe renal cell carcinoma, collecting duct carcinoma with medullary carcinoma of the kidney, and unclassified renal cell carcinoma.

SWISS:
P40337

Gene ID:
7428

Database links:

Entrez Gene: 7428 Human

Entrez Gene: 22346 Mouse

Omim: 608537 Human

SwissProt: P40337 Human

SwissProt: P40338 Mouse

Unigene: 517792 Human

Unigene: 607789 Human

Unigene: 29407 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

von Hippel-Lindau是一種腫瘤抑制因子，在細(xì)胞對(duì)氧的感受過程中發(fā)揮關(guān)鍵作用，VHL蛋白除了調(diào)節(jié)血管生成外還在調(diào)節(jié)細(xì)胞的生長和生存、對(duì)調(diào)節(jié)細(xì)胞周期、細(xì)胞凋亡和細(xì)胞外基質(zhì)方面起重要作用。