Anti-Von Hippel Lindau 抗体 (ab89643)

製品の概要

• 製品名Anti-Von Hippel Lindau antibody
  Von Hippel Lindau 一次抗体 製品一覧
• 製品の詳細
  Mouse polyclonal to Von Hippel Lindau
• 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

  Full length human Von Hippel Lindau protein, amino acids 1-172 (ENSP00000344757)

• ポジティブ･コントロール
  • Human placenta tissue lysate, Von Hippel Lindau protein transfected 293T cell lysate.

製品の特性

• 製品の状態Liquid
• 保存方法Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
• バッファーPreservative: None
  Constituents: 1X PBS, pH 7.2
• Concentration information loading...
• 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
  • Cell Biology
  • Cell Cycle
  • Cell Cycle Inhibitors
  • Other

  • Cell Biology
  • Proteolysis / Ubiquitin
  • Proteasome / Ubiquitin
  • Proteasome

  • Cell Biology
  • Proteolysis / Ubiquitin
  • Proteasome / Ubiquitin
  • Ubiquitin

  • Cell Biology
  • Cell Cycle
  • Cell differentiation

  • Epigenetics and Nuclear Signaling
  • Transcription
  • Polymerase associated factors
  • Pol II Transcription
  • Other

  • Epigenetics and Nuclear Signaling
  • Transcription
  • Co-factors

  • Cancer
  • Cell cycle
  • Cell differentiation

  • Cancer
  • Oncoproteins/suppressors
  • Tumor suppressors
  • Other

  • Cancer
  • Cancer Metabolism
  • Response to hypoxia

  • Metabolism
  • Pathways and Processes
  • Cofactors, Vitamins / minerals
  • Co-factors

  • Metabolism
  • Pathways and Processes
  • Metabolism processes
  • Hypoxia

  • Metabolism
  • Types of disease
  • Cancer

関連製品

• Compatible Secondaries
  • Goat Anti-Mouse IgG H&L (Alexa Fluor® 488) (ab150113)
  • Goat Anti-Mouse IgG H&L (HRP) (ab205719)
• Positive Controls
  • Human placenta tissue lysate - total protein (ab29745)

アプリケーション

ターゲット情報

• 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.

• Target information above from: UniProt accession P40337 The UniProt Consortium
  The Universal Protein Resource (UniProt) in 2010
  Nucleic Acids Res. 38:D142-D148 (2010) .

  Information by UniProt
• 参照データベース
  • Entrez Gene: 7428 Human
  • Omim: 608537 Human
  • SwissProt: P40337 Human
  • Unigene: 517792 Human
  • Unigene: 607789 Human
• 別名
  • Elongin binding protein antibody
  • G7 protein antibody
  • HRCA 1 antibody

  • HRCA1 antibody
  • Protein G7 antibody
  • pVHL antibody
  • RCA 1 antibody
  • RCA1 antibody
  • VHL 1 antibody
  • VHL antibody
  • VHL_HUMAN antibody
  • VHL1 antibody
  • VHLH antibody
  • Von Hippel Lindau disease tumor suppressor antibody
  • von Hippel Lindau syndrome antibody
  • von Hippel Lindau tumor suppressor antibody
  • Von Hippel Lindau tumor suppressor, E3 ubiquitin protein ligase antibody
  • Von Hippel-Lindau disease tumor suppressor antibody

  see all