The von Hippel-Lindau tumor suppressor protein

Current Opinion in Genetics and Development

Volumn 11, Issue 1, 2001, Pages 27-34

  Ivan, Mircea ^a   Kaelin, William G ^a  

^a BRIGHAM AND WOMEN S HOSPITAL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHAPERONIN; GLUCOSE TRANSPORTER; HYPOXIA INDUCIBLE FACTOR 1ALPHA; PHOSPHOGLYCERATE KINASE; TRANSCRIPTION FACTOR; VASCULOTROPIN;

ALPHA CHAIN; CELLULAR DISTRIBUTION; CONJUGATION; CRYSTAL STRUCTURE; DNA SEQUENCE; EXTRACELLULAR MATRIX; GENETIC CODE; GENETIC TRANSCRIPTION; HEMANGIOBLASTOMA; HUMAN; PHEOCHROMOCYTOMA; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN STRUCTURE; PROTEIN TARGETING; REVIEW; SEQUENCE HOMOLOGY; TRANSCRIPTION REGULATION; TUMOR SUPPRESSOR GENE; VON HIPPEL LINDAU DISEASE;

ANIMALS; ARYL HYDROCARBON RECEPTOR NUCLEAR TRANSLOCATOR; CELL CYCLE; CELL CYCLE PROTEINS; CHAPERONINS; CULLIN PROTEINS; DNA-BINDING PROTEINS; EXTRACELLULAR MATRIX; GENES, TUMOR SUPPRESSOR; HIPPEL-LINDAU DISEASE; HYPOXIA-INDUCIBLE FACTOR 1; HYPOXIA-INDUCIBLE FACTOR 1, ALPHA SUBUNIT; ISOENZYMES; LIGASES; NUCLEAR PROTEINS; PROTEIN KINASE C; PROTEINS; RECEPTORS, ARYL HYDROCARBON; TRANSCRIPTION FACTORS; TRANSCRIPTION, GENETIC; TUMOR SUPPRESSOR PROTEINS; UBIQUITIN-PROTEIN LIGASES; UBIQUITINS; VON HIPPEL-LINDAU TUMOR SUPPRESSOR PROTEIN;

EID: 0035253381     PISSN: 0959437X     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0959-437X(00)00152-0     Document Type: Review

References (79)

1. A second major native von Hippel-Lindau gene product, initiated from an internal translation start site, functions as a tumor suppressor
2. pVHL19 is a biologically active product of the von Hippel-Lindau gene arising from internal translation initiation
3. Alternate choice of initiation codon produced a biologically active product of the von Hippel Lindau gene with tumor suppressor activity
4. Inhibition of transcriptional elongation by the VHL tumor suppressor protein
5. Binding of the von Hippel-Lindau tumor suppressor protein to elongin B and C
6. The von Hippel-Lindau tumor-suppressor gene product forms a stable complex with human CUL-2, a member of the Cdc53 family of proteins
7. Regulation of hypoxia-inducible mRNAs by the von Hippel-Lindau protein requires binding to complexes containing elongins B/C and Cul2
8. Rbx1, a component of the VHL tumor suppressor complex and SCF ubiquitin ligase
9. Studying interactions of four proteins in the yeast two-hybrid system: Structural resemblance of the pVHL/elongin BC/hCUL-2 complex with the ubiquitin ligase complex SKP1/cullin/F-box protein
10. Synthetic peptides define critical contacts between elongin C, elongin B, and the von hippel-lindau protein
11. Cellular proteins that bind the von Hippel-Lindau disease gene product: Mapping of binding domains and the effect of missense mutations
12. SKP1 connects cell cycle regulators to he ubiquitin proteolysis machinery through a novel motif, the F-box
13. Cdc53p acts in concert with Cdc4p and Cdc34p to control the G1-to-S-phase transition and identifies a conserved family of proteins
14. SCF and Cullin/Ring H2-based ubiquitin ligases
15. Ubiquitin-mediated proteolysis: Biological regulation via destruction
16. Structure of the VHL-ElonginC-elonginB complex: Implications for VHL tumor suppressor function
17. The von Hippel-Lindau tumor suppressor protein is a component of an E3 ubiquitin-protein ligase activity
18. Identification of the von Hippel-Lindau tumor-suppressor protein as part of an active E3 ubiquitin ligase complex
19. Up-regulation of vascular endothelial growth factor and its receptors in von Hippel-Lindau disease-associated and sporadic hemangioblastomas
20. Polycythemia: Mechanisms and management
21. Coexpression of erythropoietin and vascular endothelial growth factor in nervous system tumors associated with von Hippel-Lindau tumor suppressor gene loss of function
22. Reversion of deregulated expression of vascular endothelial growth factor in human renal carcinoma cells by von Hippel-Lindau tumor suppressor protein
23. Post-transcriptional regulation of vascular endothelial growth factor mRNA by the VHL tumor suppressor gene product
24. Negative regulation of hypoxia-inducible genes by the von Hippel-Lindau protein
25. Putative control of angiogenesis in hemangioblastomas by the von Hippel-Lindau tumor suppressor gene
26. The von Hippel-Lindau gene product is necessary for oxgyen-dependent proteolysis of hypoxia-inducible factor a subunits
27. Hypoxia inducible factor-alpha binding and ubiquitylation by the von Hippel-Lindau tumor suppressor protein
28. Ubiquitination of HIF requires direct binding to the von Hippel-Lindau protein beta domain
29. Mechanism of regulation of the hypoxia-inducible factor-1alpha by the von Hippel-Lindau tumor suppressor protein
30. Characterization of an oxygen/redox-dependent degradation domain of hypoxia-inducible factor alpha (HIF-alpha) proteins
31. Activation of HIF1α ubiquitination by a reconstituted von Hippel-Lindau tumor suppressor complex
32. The Elongin BC complex interacts with the conserved SOCS-box motif present in members of the SOCS, ras, WD-40 repeat, and ankyrin repeat families
33. Suppressor of cytokine signaling-1 inhibits VAV function through protein degradation
34. The conserved SOCS box motif in suppressors of cytokine signaling binds to elongins B and C and may couple bound proteins to proteasomal degradation
35. Evolution and function of ubiquitin-like protein-conjugation systems
36. The Rbx1 subunit of SCF and VHL E3 ubiquitin ligase activates Rub1 modification of cullins Cdc53 and Cul2
37. Conjugation of the ubiquitin-like protein NEDD8 to cullin-2 is linked to von Hippel-Lindau tumor suppressor function
38. The von Hippel-Lindau tumor suppressor gene product promotes, but is not essential for, NEDD8 conjugation to cullin-2
39. bTrCP dependent ubiquitination of IkBa
40. Conjugation of Nedd8 to CUL1 enhances the ability of the ROC1-CUL1 complex to promote ubiquitin polymerization
41. A Nedd8 conjugation pathway is essential for proteolytic targeting of p27Kip1 by ubiquitination
42. Modification of cullin-1 by ubiquitin-like protein Nedd8 enhances the activity of SCF(skp2) toward p27(kip1)
43. The von Hippel-Lindau tumor suppressor gene product interacts with Sp1 to repress vascular endothelial growth factor promoter activity
44. Elongin (SIII): A multisubunit regulator of elongation by RNA polymerase II
45. von Hippel-Lindau protein induces hypoxia-regulated arrest of tyrosine hydroxylase transcript elongation in pheochromocytoma cells
46. Regulation of gene expression for tyrosine hydroxylase in oxygen sensitive cells by hypoxia
47. Nuclear/cytoplasmic localization of the von Hippel-Lindau tumor suppressor gene product is determined by cell density
48. Transcription-dependent nuclear-cytoplasmic trafficking is required for the function of the von Hippel-Lindau tumor suppressor protein
49. Ran-mediated nuclear export of the von Hippel-Lindau tumor suppressor protein occurs independently of its assembly with cullin-2
50. Moving protein heads for breakdown
51. Binding of elongin A or a von Hippel-Lindau peptide stabilizes the structure of yeast elongin C
52. Formation of the VHL-elongin BC tumor suppressor complex is mediated by the chaperonin TriC
53. Elongin BC complex prevents degradation of von Hippel-Lindau tumor suppressor gene products
54. The von Hippel-Lindau tumor suppressor protein is required for proper assembly of an extracellular fibronectin matrix
55. von Hippel-Lindau gene-mediated growth suppression and induction of differentiation in renal cell carcinoma cells grown as multicellular tumor spheroids
56. The von Hippel-Lindau tumor suppressor gene inhibits hepatocyte growth factor/scatter factor-induced invasion and branching morphogenesis in renal carcinoma cells
57. Regulation of the urokinase-type plasminogen activator system by the von Hippel-Lindau tumor suppressor gene
58. The von Hippel-Lindau tumor suppressor gene is required for cell cycle exit upon serum withdrawal
59. Recombinant adenovirus expressing Von Hippel-Lindau-mediated cell cycle arrest is associated with the induction of cyclin-dependent kinase inhibitor p27Kip1
60. Transforming growth factor alpha is a target for the von Hippel-Lindau tumor suppressor
61. Requirement for the von Hippel-Lindau tumor suppressor gene for functional epidermal growth factor receptor blockade by monoclonal antibody C225 in renal cell carcinoma
62. The von Hippel-Lindau gene product inhibits vascular permeability factor/vascular endothelial growth factor expression in renal cell carcinoma by blocking protein kinase C pathways
63. Direct interaction of the beta-domain of VHL tumor suppressor protein with the regulatory domain of atypical PKC isotypes
64. Inhibition of insulin-like growth factor-I-mediated cell signaling by the von Hippel-Lindau gene product in renal cancer
65. Comparative sequence analysis of the VHL tumor suppressor gene
66. Characterization of the VHL tumor suppressor gene product: Localization, complex formation, and the effect of natural inactivating mutations
67. Cloning and characterization of a mouse gene with homology to the human von Hippel-Lindau disease tumor suppressor gene: Implications for the potential organization of the human von Hippel-Lindau disease gene
68. Tracheal development and the von Hippel-Lindau tumor suppressor homolog in Drosophila
69. Defective placental vasculogenesis causes embryonic lethality in VHL-deficient mice
70. Haemangioblastoma of the central nervous system in von Hippel-Lindau disease
71. von Hippel-Lindau gene deletion detected in the stromal cell component of a cerebellar hemangioblastoma associated with von Hippel-Lindau disease
72. Loss of heterozygosity and somatic mutations of the VHL tumor suppressor gene in sporadic cerebellar hemangioblastomas
73. Up-regulation of vascular endothelial growth factor in stromal cells of hemangioblastomas is correlated with up-regulation of the transcription factor HRF/HIF-2alpha
74. Renal cysts, renal cancer and von Hippel-Lindau disease
75. Biology of disease: Biology of polycystic kidney disease
76. Polycystic kidney disease 1: Identification and analysis of the primary defect
77. Vascular permeability factor/vascular endothelial growth factor and the significance of microvascular hyperpermeability in angiogenesis
78. The hypoxia-responsive transcription factor EPAS1 is essential for catecholamine homeostasis and protection against heart failure during embryonic development
79. Cancer, Many vessels, faulty gene