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  4. Recombinant Mouse Von Hippel-Lindau disease tumor suppressor (Vhl)

Recombinant Mouse Von Hippel-Lindau disease tumor suppressor (Vhl)

  • 货号：
    CSB-YP025852MO
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 货号：
    CSB-EP025852MO
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 货号：
    CSB-EP025852MO-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 货号：
    CSB-BP025852MO
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 货号：
    CSB-MP025852MO
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
  • Uniprot No.：
  • 别名：
    Vhl; Vhlh; von Hippel-Lindau disease tumor suppressor; pVHL
  • 种属：
    Mus musculus (Mouse)
  • 蛋白长度：
    Full length protein
  • 表达区域：
    1-181
  • 氨基酸序列
    MPRKAASPEE AAGEPGPEEM EAGRPRPVLR SVNSREPSQV IFCNRSPRVV LPLWLNFDGE PQPYPILPPG TGRRIHSYRG HLWLFRDAGT HDGLLVNQTE LFVPSLNVDG QPIFANITLP VYTLKERCLQ VVRSLVKPEN YRRLDIVRSL YEDLEDYPSV RKDIQRLSQE HLESQHLEEE P
  • 蛋白标签：
    Tag type will be determined during the manufacturing process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 产品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 复溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    Involved in the ubiquitination and subsequent proteasomal degradation via the von Hippel-Lindau ubiquitination complex. Seems to act as a 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.
  • 基因功能参考文献：
    1. VHL role in the clear cell renal cell carcinoma PMID: 28593993
    2. Data show that 100% of von Hippel-Lindau tumor suppressor protein (Vhl) / polybromo 1 protein (Pbrm1) knockout mice developed renal cancers by 20 months of age. PMID: 29229903
    3. VHL-HIF-glycolysis axis is essential for the late-stage maturation and function of ILC2s via targeting IL-33-ST2 pathway. PMID: 29452935
    4. Oxidative stress upregulates pVHL expression to induce PLN degradation in failing hearts. PMID: 29068413
    5. Loss of VHL could be driving tumour cell dissemination through stabilization of HIF-1alpha in a murine renal-cell carcinoma model. Overexpression of HIF-1alpha-regulated genes that are upregulated in our VHL knockout cells signifies an aggressive form of clear-cell renal-cell carcinoma. PMID: 27358011
    6. We conditionally targeted Bap1 and Pbrm1 (with Vhl) in the mouse using several Cre drivers.Sglt2 and Villin proximal convoluted tubule drivers failed to cause tumorigenesis, challenging the conventional notion of Clear cell renal cell carcinoma (ccRCC)origins PMID: 28473526
    7. dual inactivation of Vhl with either Bap1 or Pbrm1 results in faithful genetically engineered murine models of clear cell renal cell carcinoma (ccRCC). PMID: 28765116
    8. HIF-1alpha-dependent, HIF-2alpha-independent angiogenesis and constitutive diuresis is caused by Vhl deletion in renal epithelia PMID: 27528422
    9. HIF-1alpha/miR-210 pathway is strongly activated in VHL mutated paragangliomas, weakly activated in SDHx mutated PGLs, and not activated in VHLdel- and SDHxwt/VHLwt-PGLs. PMID: 28036268
    10. kidney-specific deletion of Vhl and Pbrm1, but not either gene alone, results in bilateral, multifocal, transplantable clear cell kidney cancers. PMID: 28329682
    11. Codeletion of VHL together with HIF2A but not with HIF1A led to apparently normal kidneys, and the animals reached normal age but were anemic because of low erythropoietin levels. Stromal deletion of HIF2A or HIF1A alone did not affect kidney development. PMID: 28527294
    12. High VHL expression is associated with liver fibrosis. PMID: 28118605
    13. Study demonstrated that mice with a loss of one allele of the VHL gene exhibited normal phenotypes without evidence of predisposition to the development of CNS-hemangioblastomas. Heterozygous VHL +/- mice exhibited the neuroprotective response to acute cerebral ischemia/reperfusion injury due to enhanced angiogenesis by HIF-dependent regulation and HIF-independent Twist 1 signaling. PMID: 28574654
    14. These results support the idea that the development of a full-blown VHL disease phenotype requires inactivation of the VHL gene not only in the tumor proper, but also in the stromal compartment. PMID: 27733136
    15. pVHL has a pivotal role in bleomycin-induced pulmonary fibrosis. PMID: 26488390
    16. These findings reveal VHL-HIF-mediated metabolic compartmentalization in the developing heart and the connection between metabolism and myocardial differentiation. PMID: 27997827
    17. Loss of Vhl in mesenchymal progenitors of the limb bud caused severe fibrosis of the synovial joints and formation of aggressive masses with histologic features of mesenchymal tumors. PMID: 26348575
    18. These data suggested that intestinal epithelial cells were injured after IM treatment through the pVHL overexpression-induced degradation of collagen I or HIF-1alpha. PMID: 26551465
    19. Vhl and Kif3a deletion accelerates renal cyst formation PMID: 25788526
    20. maintains the stability and suppressive function of Foxp3+ T cells via regulation of HIF-1a pathway PMID: 26084024
    21. Conditional inactivation of the mouse von Hippel-Lindau tumor suppressor gene results in wide-spread hyperplastic, inflammatory and fibrotic lesions in the kidney. PMID: 25023703
    22. Deletion of the Vhl gene causes sympathoadrenal cell death and impairs chemoreceptor-mediated adaptation to hypoxia. PMID: 25385837
    23. Molecular dynamics of hif-1alpha and VHL may determine the success of antineoplastic strategies in hypoxia-reoxygenation as predicted by computational modeling. PMID: 25338163
    24. Loss of Vhl in adult joint cartilage is associated with earlier dysregulation of cartilage homeostasis, characterized by increased chondrocyte apoptosis, compromised chondrocyte autophagy and accelerated age-related and surgery-induced OA development. PMID: 24999110
    25. Both Vhl and Bap1 are required for kidney function. Even when Vhl is inactivated in multipotent nephron progenitor cells, Vhl loss is insufficient for renal tumorigenesis. PMID: 25359211
    26. VHL deficiency perturbs pancreas endocrine homeostasis in mice. PMID: 23977255
    27. data suggest that the VHL-mediated signaling in osteochondral progenitor cells plays a critical role in bone remodeling at postnatal/adult stages through coupling osteogenesis and angiogenesis PMID: 23999831
    28. VHL regulates bone morphogenesis as its loss considerably alters size, shape and overall development of the skeletal elements. PMID: 24972088
    29. A key role for pVHL in mediating oriented cell division and faithful mitotic checkpoint function in the renal epithelium PMID: 24362914
    30. Disruption of Vhl led to a HIF-2alpha-dependent decrease in cholesterol hydroxylases involved in the conversion of cholesterol to bile acids. PMID: 24421394
    31. HIFa1 plays an essential role in the pathogenesis of renal cell carcinoma under normoxic conditions, through the loss of the Von Hippel-Lindau gene PMID: 24344197
    32. Conditional VHL gene deletion causes hypoglycemic death associated with disproportionately increased glucose uptake by hepatocytes through an upregulated IGF-I receptor. PMID: 23874892
    33. the HIF-mediated renoprotection in VHL-KO mice was associated with activated glycolysis, cellular glucose uptake and utilization, autophagy, vasodilation, and proton removal PMID: 23970125
    34. Data indicate that microtubule dynamics are altered in primary cells derived from Vhl(M1L/M1L) mice to an extent similar to that seen following complete loss of pVHL function. PMID: 23541568
    35. Inducible, Pax8-rtTA-based deletion of VHL leads to organ-specific expression of epithelial HIF and erythropoietin in liver and kidney without causing pathological changes. PMID: 23384425
    36. deletion of Vhl shifts the phenotype of juxtaglomerular cells from a renin- to erythropoietin-secreting cell type, presumably in response to HIF-2 accumulation PMID: 23393316
    37. The data showed that Von Hippel-Lindau protein-dependent regulation of Hif1a in the retinal pigment epithelium is essential for normal retinal pigment epithelial and iris development, ocular growth and vascular development in the anterior chamber. PMID: 22627278
    38. VHL appears to specifically repress HIF-2alpha in renal epithelia. PMID: 22299048
    39. Runx2 stabilizes HIF-1alpha by binding to ODDD to block the interaction between von Hippel-Lindau protein and HIF-1alpha. PMID: 22351759
    40. Astrocytic Vhl deletion induced accelerated hyaloidal regression and subsequent massive secondary outgrowth. PMID: 22084310
    41. we show that pVHL, a tumor suppressor protein that has been implicated in the pathogenesis of renal cell carcinoma (RCC), plays an important role in regulation of cytokinesis. PMID: 21652636
    42. Deficiency of Vhl in the thick ascending limb attenuated proximal tubular injury and preserved its function following ischemia-reperfusion, which may be partially a result of enhanced expression of glycolytic enzymes and lactate metabolism. PMID: 21921145
    43. Vhl gene inactivation rapidly resulted in a marked splenomegaly and skin erythema, accompanied by renal and hepatic induction of the erythropoietin (Epo) gene, indicative of the in vivo activation of the oxygen sensing HIF pathway. PMID: 21811636
    44. cooperation of VHL and EAF2 may be critical for angiogenic regulation of the liver and prostate, and concurrent loss of these two tumor suppressors may result in a pro-angiogenic phenotype. PMID: 21638067
    45. Tissue-specific knockdown of von Hippel-Lindau protein (VHL) activates HIF transcription factors in normoxic conditions in the retina. PMID: 20817091
    46. Myb-binding protein p160 is a novel target of the von Hippel-Lindau tumor suppressor. PMID: 21386990
    47. Critical role for VHL in beta-cell function and mass, and erythropoietin administration improved beta-cell function making it potential strategy for diabetes treatment. PMID: 21242957
    48. VHL deletion impairs mammary alveologenesis but is not sufficient for mammary tumorigenesis PMID: 20382704
    49. Data suggest that podocyte von Hippel-Lindau protein is required for normal maintenance of podocytes, basment membrane collagen {alpha}1{alpha}2{alpha}1(IV) deposition and ultrastructure, neuroglobin experssion and glomerular barrier properties. PMID: 20522651
    50. Data show that VHL loss leads to senescence under atmospheric conditions (21% O(2)). PMID: 20679489

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  • 亚细胞定位：
    Cytoplasm. Membrane; Peripheral membrane protein. Nucleus.
  • 蛋白家族：
    VHL family
  • 数据库链接：

    KEGG: mmu:22346

    STRING: 10090.ENSMUSP00000039418

    UniGene: Mm.29407