货号：MABC14

品牌：Merck Millipore

规格：100Ug

目录价：￥5666.00

市场价格：￥4816.10

会员价格：￥4532.80

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-VHL Antibody, clone 11E12 | MABC14 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: ~ 24 kDa observed MW. VHL SUMOylation may result in high MW bands (Cai, 2010). View All» Immunogen: Purified human VHL View All» Clone: 11E12 View All» Isotype: IgG1κ View All» Background Information: von Hippel-Lindau protein (VHL) is most widely recognized as belonging to an E3 ubiquitin ligase complex that specifically targets hypoxia-inducible factors (HIFs) for the purpose of polyubiquitination and proteolytic degradation. Within this complex, VHL acts as a target recruitment subunit for HIFα and its subsequent ubiquitination. The complex also contains proteins such as B and C elongins, Rbx1 and Cul2. Inactivation of VHL has been linked to renal cell carcinoma development, therefore suggesting that VHL plays a role as a tumor suppressor. VHL expression has been observed in both fetal and adult kidney and brain tissues. Defects in VHL expression are causal to renal cell carcinoma type 1 (RCC1), erythrocytosis familial type 2 (ECYT2), von Hippel-Lindau disease (VHLD), and pheochromocytoma. View All» Species Reactivity:

• Mouse

• Human
  View All» Species Reactivity Note: Demonstrated to react with mouse and human. View All» Application Notes: Immunoprecipitation: It has been shown that this VHL clone will immunoprecipitate VHL from HEK 293 cells (Shoenfeld, 1998). View All» Control: Mouse fetal brain tissue lysate View All» Quality Assurance: Evaluated by Western Blot in mouse fetal brain tissue lysate.Western Blot Analysis: 1 µg/mL of the antibody detected VHL in 10 µg of mouse fetal brain tissue lysate. View All» Purification Method: Protein G View All» Presentation: Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: P40337 View All» Entrez Gene Number: NP_000542 View All» Gene Symbol:
  • VHL

  • VHL1

  • HRCA1

  • RCA1

  • pVHL
    View All» Alternate Names:
    • von Hippel-Lindau disease tumor suppressor

    • Protein G7

    • elongin binding protein

    • von Hippel-Lindau syndrome

    • pVHL
      View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
      • Western Blotting

      • Immunoprecipitation
        View All» Entrez Gene Summary: 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]. View All» UniProt Summary: 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. PATHWAY: Protein modification; protein ubiquitination.SUBUNIT STRUCTURE: Component of the VCB (VHL-Elongin BC-CUL2) complex; this complex acts as a ubiquitin-ligase E3 and directs proteosome-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 RNF139 and UBP33. Interacts with 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. 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.DOMAIN: The Elongin BC complex binding domain is also known as BC-box with the consensus [APST]-L-x(3)-C-x(3)-[AILV].INVOLVEMENT IN IDSEASE: Defects in VHL are a cause of pheochromocytoma [MIM:171300]. The pheochromocytomas are catecholamine-producing, chromaffin tumors that arise in the adrenal medulla in 90% of cases. In the remaining 10% of cases, they develop in extra-adrenal sympathetic ganglia and may be referred to as "paraganglioma." Pheochromocytoma usually presents with hypertension. Approximately 10% of pheochromocytoma is hereditary. The genetic basis for most cases of non-syndromic familial pheochromocytoma is unknown. 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 type 1 (RCC1) [MIM:144700]; also known as hypernephroma or adenocarcinoma of kidney. Familial renal cell carcinoma syndromes form a group of diseases characterized by a predisposition to development of renal cell carcinomas (RCCs) with various histological subtypes. View All» Product Name: Anti-VHL, clone 11E12 View All» Concentration: 1 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
        © 金山科研平台是专业的授权总代理区域代理经销平台。
        © 如需询价，请加客服QQ：1749072012 、客服微信：jinshanbio，或发送邮件到1749072012@qq.com
        © 平台为生命科学研究相关领域提供一站式耗材试剂仪器解决方案和采购服务，数据资源基于CC协议。
        © 本文地址：https://16ao.com/thread-132810.htm
        © Merck Millipore代理MABC14 Anti-VHL Antibody, clone 11E12;store at +2℃ to +8℃，产品报价联系微信jinshanbio