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
製品の概要
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製品名
Anti-Von Hippel Lindau/VHL antibody
Von Hippel Lindau/VHL 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to Von Hippel Lindau/VHL -
由来種
Rabbit -
アプリケーション
適用あり: WBmore details -
種交差性
交差種: Human -
免疫原
Recombinant full length protein corresponding to Human Von Hippel Lindau/VHL.
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ポジティブ・コントロール
- HeLa nuclear extract
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特記事項
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.
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製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
バッファー
pH: 7.9 -
Concentration information loading...
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精製度
Protein A purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
研究分野
関連製品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab28434の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
アプリケーション | Abreviews | 特記事項 |
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WB |
Use at an assay dependent concentration.
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特記事項 |
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WB
Use at an assay dependent concentration. |
ターゲット情報
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機能
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. -
組織特異性
Expressed in the adult and fetal brain and kidney. -
パスウェイ
Protein modification; protein ubiquitination. -
関連疾患
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. -
ドメイン
The Elongin BC complex binding domain is also known as BC-box with the consensus [APST]-L-x(3)-C-x(3)-[AILV]. -
細胞内局在
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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参照データベース
- Entrez Gene: 7428 Human
- Omim: 608537 Human
- SwissProt: P40337 Human
- Unigene: 517792 Human
- Unigene: 607789 Human
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別名
- Elongin binding protein antibody
- G7 protein antibody
- HRCA 1 antibody
see all
データシートおよび資料
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Datasheet download
参考文献 (4)
ab28434 は 4 報の論文で使用されています。
- 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