Anti-Von Hippel Lindau/VHL抗体(ab28434)
Key features and details
- Rabbit polyclonal to Von Hippel Lindau/VHL
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
概述
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产品名称
Anti-Von Hippel Lindau/VHL抗体
参阅全部 Von Hippel Lindau/VHL 一抗 -
描述
兔多克隆抗体to Von Hippel Lindau/VHL -
宿主
Rabbit -
经测试应用
适用于: WBmore details -
种属反应性
与反应: Human -
免疫原
Recombinant full length protein corresponding to Human Von Hippel Lindau/VHL.
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阳性对照
- HeLa nuclear extract
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常规说明
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
存储溶液
pH: 7.9 -
Concentration information loading...
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纯度
Protein A purified -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab28434于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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WB |
Use at an assay dependent concentration.
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说明 |
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WB
Use at an assay dependent concentration. |
靶标
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功能
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. -
组织特异性
Expressed in the adult and fetal brain and kidney. -
通路
Protein modification; protein ubiquitination. -
疾病相关
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 erythrocytosis familial 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. -
结构域
The Elongin BC complex binding domain is also known as BC-box with the consensus [APST]-L-x(3)-C-x(3)-[AILV]. -
细胞定位
Cytoplasm. Membrane. Nucleus. Found predominantly in the cytoplasm and with less amounts nuclear or membrane-associated and Cytoplasm. Nucleus. Equally distributed between the nucleus and the cytoplasm but not membrane-associated. - Information by UniProt
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数据库链接
- Entrez Gene: 7428 Human
- Omim: 608537 Human
- SwissProt: P40337 Human
- Unigene: 517792 Human
- Unigene: 607789 Human
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别名
- Elongin binding protein antibody
- G7 protein antibody
- HRCA 1 antibody
see all
数据表及文件
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Datasheet download
文献 (4)
ab28434 被引用在 4 文献中.
- Devine RD et al. Increased hypoxia-inducible factor-1a in striated muscle of tumor-bearing mice. Am J Physiol Heart Circ Physiol 312:H1154-H1162 (2017). PubMed: 28341633
- Shmueli MD et al. Computational and experimental characterization of dVHL establish a Drosophila model of VHL syndrome. PLoS One 9:e109864 (2014). PubMed: 25310726
- Gammon L et al. Sub-sets of cancer stem cells differ intrinsically in their patterns of oxygen metabolism. PLoS One 8:e62493 (2013). WB ; Human . PubMed: 23638097
- André H & Pereira TS Identification of an alternative mechanism of degradation of the hypoxia-inducible factor-1alpha. J Biol Chem 283:29375-84 (2008). WB ; Human . PubMed: 18694926