The von Hippel-Lindau tumor suppressor gene

Experimental Cell Research

Volumn 264, Issue 1, 2001, Pages 117-125

  Kondo, Keiichi ^a   Kaelin Jr , William G ^a  

^a BRIGHAM AND WOMEN S HOSPITAL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

GENE PRODUCT; HYPOXIA INDUCIBLE FACTOR 1; LIGASE; OXYGEN; TRANSCRIPTION FACTOR; UBIQUITIN; UNCLASSIFIED DRUG; VASCULOTROPIN; VON HIPPEL LINDAU PROTEIN;

ALLELISM; CARCINOGENESIS; CELL CYCLE; CLEAR CELL CARCINOMA; FAMILIAL CANCER; GENE CONTROL; GENE MUTATION; GENE TARGETING; GERM LINE; HUMAN; KIDNEY CARCINOMA; NONHUMAN; PHEOCHROMOCYTOMA; PRIORITY JOURNAL; REVIEW; TUMOR DIFFERENTIATION; TUMOR SUPPRESSOR GENE; VON HIPPEL LINDAU DISEASE;

EID: 0035835819     PISSN: 00144827     EISSN: None     Source Type: Journal    
DOI: 10.1006/excr.2000.5139     Document Type: Article

References (121)

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2. Zur frage der angiomatosis retinae und ihrer hirncomplikation
3. Ueber eine sehr seltene erkrankung der nethaut
4. Von Hippel-Lindau disease
5. Von Hippel-Lindau disease
6. Von-Hippel Lindau disease maps to the region of chromosome 3 associated with renal cell carcinoma
7. Identification of the von Hippel-Lindau disease tumor suppressor gene
8. Improved detection of germline mutations in the von Hippel-Lindau disease tumor suppressor gene
9. Mosaicism in von Hippel-Lindau disease: Lessons from kindreds with germline mutations identified in offspring with mosaic parents
10. Somatic mosaicism in von Hippel-Lindau disease
11. Genotype-phenotype correlations in von Hippel-Lindau disease
12. Consequences of direct genetic testing for germline mutations in the clinical management of families with multiple endocrine neoplasia type 2
13. Isolated familial pheochromocytoma as a variant ofvon Hippel-Lindau disease
14. Mutations in the RET proto-oncogene and the von Hippel-Lindau disease tumour suppressor gene in sporadic and syndromic phaeochromocytomas
15. Germline mutations in the vhl gene in patients presenting with phaeochromocytomas
16. Molecular genetic diagnosis of von Hippel-Lindau disease in familial phaeochromocytoma
17. Loss of heterozygosity and somatic mutations of the VHL tumor suppressor gene in sporadic cerebellar hemangioblastomas
18. Somatic mutations of the von Hippel-Lindau tumor supressor gene in sporadic central nervous systems hemangioblastomas
19. Molecular genetic analysis of the von Hippel-Lindau disease tumor suppressor gene in familial and sporadic cerebellar hemangioblastomas
20. Mutation of the Von Hippel-Lindau tumour suppressor gene in capillary haemangioblastomas of the central nervous system
21. Germline and somatic mutations in von Hippel-Lindau disease gene and its significance in the development of kidney cancer
22. Frequent somatic mutations and loss of heterozygosity of the von Hippel-Lindau tumor suppressor gene in primary human renal cell carcinomas
23. Somatic mutations of the von Hippel-Lindau disease tumor suppressor gene in non-familial clear cell renal carcinoma
24. Germ-line mutations in the von Hippel-Lindau tumor suppressor gene are similar to somatic von Hippel-Lindau abberations in sporadic renal cell carcinoma
25. Allelic deletions of the VHL gene detected in multiple microscopic clear cell renal lesions in von Hippel-Lindau disease patients
26. Detection of von Hippel-Lindau disease gene mutations in paraffin-embedded sporadic renal cell carcinoma specimens
27. Inactivation of the von Hippel-Lindau (VHL) tumour suppressor gene and allelic losses at chromosome arm 3p in primary renal cell carcinoma: Evidence for a VHL-independent pathway in clear cell renal tumourigenesis
28. Mutations of the VHL tumour suppressor gene in renal carcinoma
29. Silencing of the VHL tumor-suppressor gene by DNA methylation in renal carcinoma
30. Extensive mutation scanning of RET in sporadic medullary thyroid carcinoma and of RET and VHL in sporadic pheochromocytoma reveals involvement of these genes in only a minority of cases
31. Isolation and characterization of the full-length 3′ untranslated region of the human von Hippel-Lindau tumor suppressor gene
32. Identification of the promoter of the human von Hippel-Lindau disease tumor suppressor gene
33. Tracheal development and the von Hippel-Lindau tumor suppressor homolog in Drosophila
34. 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
35. Characterization of the VHL tumor suppressor gene product: Localization, complex formation, and the effect of natural inactivating mutations
36. Comparative sequence analysis of the VHL tumor suppressor gene
37. Tumor suppression by the human von Hippel-Lindau gene product
38. pVHL19 is a biologically active product of the von Hippel-Lindau gene arising from internal translation initiation
39. A second major native von Hippel-Lindau gene product, initiated from an internal translation start site, functions as a tumor suppressor
40. Alternate choice of initiation codon produces a biologically active product of the von Hippel Lindau gene with tumor suppressor activity
41. Nuclear/cytoplasmic localization of the von Hippel-Lindau tumor suppressor gene product is determined by cell density
42. Transcription-dependent nuclear-cytoplasmic trafficking is required for the function of the von Hippel-Lindau tumor suppressor protein
43. Immunostaining of the von Hippel-Lindau gene product (pVHL) in normal and neoplastic human tissues
44. Expression pattern of the von Hippel-Lindau protein in human tissues
45. Ran-mediated nuclear export of the von Hippel-Lindau tumor suppressor protein occurs independently of its assembly with cullin-2
46. Inhibition of transcriptional elongation by the VHL tumor suppressor protein
47. Binding of the von Hippel-Lindau tumor suppressor protein to elongin B and C
48. Cellular proteins that bind the von Hippel-Lindau disease gene product: Mapping of binding domains and the effect of missense mutations
49. Elongin (SIII): A multisubunit regulator of elongation by RNA polymerase II
50. Synthetic peptides define critical contacts between elongin C, elongin B, and the von Hippel-Lindau protein
51. Structure of the VHL-ElonginC-elonginB complex: Implications for VHL tumor suppressor function
52. 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
53. Regulation of hypoxia-inducible mRNAs by the von Hippel-Lindau protein requires binding to complexes containing Elongins B/C and Cul2
54. The von Hippel-Lindau tumor-suppressor gene product forms a stable complex with human CUL-2, a member of the Cdc53 family of proteins
55. 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
56. Cdc53 targets phosphorylated G1 cyclins for degradation by the ubiquitin proteolytic pathway
57. Cdc53p acts in concert with Cdc4p and Cdc34p to control the G1-to-S-phase transition and identifies a conserved family of proteins
58. Cell cycle: Cull and destroy
59. SKP1 connects cell cycle regulators to he ubiquitin proteolysis machinery through a novel motif, the F-box
60. SCF and cullin/ring H2-based ubiquitin ligases
61. Rbx1, a component of the VHL tumor suppressor complex and SCF ubiquitin ligase
62. The Rbx1 subunit of SCF and VHL E3 ubiquitin ligase activates Rub1 modification of cullins Cdc53 and Cul2
63. Reconstitution of G1 cyclin ubiquitination with complexes containing SCFGrr1 and rbx1
64. ROC1, a homolog of APC11, represents a family of cullin partners with an associated ubiquitin ligase activity
65. Recruitment ofa ROC1-CUL1 ubiquitin ligase by Skp1 and HOS to catalyze the ubiquitination of lκBα
66. Conjugation of Nedd8 to CUL1 enhances the ability of the ROC1-CUL1 complex to promote ubiquitin polymerization
67. Identification of the von Hippel-Lindau tumor-suppressor protein as part of an active E3 ubiquitin ligase complex
68. The von Hippel-Lindau tumor suppressor protein is a component of an E3 ubiquitin-protein ligase activity
69. Polycythemia: Mechanisms and management
70. Post-transcriptional regulation of vascular endothelial growth factor mRNA by the VHL tumor suppressor gene product
71. Negative regulation of hypoxia-inducible genes by the von Hippel-Lindau protein
72. Reversion of deregulated expression of vascular endothelial growth factor in human renal carcinoma cells by von Hippel-Lindau tumor suppressor protein
73. Putative control of angiogenesis in hemangioblastomas by the von Hippel-Lindau tumor suppressor gene
74. HIF-1 and human disease: One highly involved factor
75. The von Hippel-Lindau gene product is necessary for oxgyen-dependent proteolysis of hypoxia-inducible factor α subunits
76. Ubiquitination of HIF requires direct binding to the von Hippel-Lindau protein beta domain
77. Activation of HIF1a ubiquitination by a reconstituted von Hippel-Lindau tumor suppressor complex
78. Mechanism of regulation of the hypoxia-inducible factor-1alpha by the von Hippel-Lindau tumor suppressor protein
79. Hypoxia inducible factor-alpha binding and ubiquitylation by the von Hippel-Lindau tumor suppressor protein
80. The von Hippel-Lindau tumor suppressor protein is required for proper assembly of an extracellular fibronectin matrix
81. Elongin BC complex prevents degradation of von Hippel-Lindau tumor suppressor gene products
82. Formation of the VHL-elongin BC tumor suppressor complex is mediated by the chaperonin TRiC
83. Endoplasmic reticulum degradation: Reverse protein flow of no return
84. Protective function of von Hippel-Lindau protein against impaired protein processing in renal carcinoma cells
85. The von Hippel-Lindau tumor suppressor gene inhibits hepatocyte growth factor/scatter factor-induced invasion and branching morphogenesis in renal carcinoma cells
86. Regulation of the urokinase-type plasminogen activator system by the von Hippel-Lindau tumor suppressor gene
87. An important von Hippel-Lindau tumor suppressor domain mediates Sp1-binding and self-association
88. The von Hippel-Lindau tumor suppressor gene product interacts with Sp1 to repress vascular endothelial growth factor promoter activity
89. Direct interaction of the beta-domain of VHL tumor suppressor protein with the regulatory domain of atypical PKC isotypes
90. 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
91. Inhibition of insulin-like growth factor-I-mediated cell signaling by the von Hippel-Lindau gene product in renal cancer
92. Drosophila von Hippel-Lindau tumor suppressor complex possesses E3 ubiquitin ligase activity
93. Defective placental vasculogenesis causes embryonic lethality in VHL-deficient mice
94. Hypoxia and human placental development
95. Expression, ontogeny, and regulation of hypoxia-inducible transcription factors in the human placenta
96. Haemangioblastoma of the central nervous system in von Hippel-Lindau disease
97. Expression of growth factors and growth factor receptors in capillary hemangioblastoma
98. Up-regulation of vascular endothelial growth factor in stromal cells of hemangioblastomas is correlated with up-regulation of the transcription factor HRF/HIF-2alpha
99. Von Hippel-Lindau gene deletion detected in the stromal cell component of a cerebellar hemangioblastoma associated with von Hippel-Lindau disease
100. Coexpression of transforming growth factor-alpha and epidermal growth factor receptor in capillary hemangioblastomas of the central nervous system
101. Up-regulation of vascular endothelial growth factor and its receptors in von Hippel-Lindau disease-associated and sporadic hemangioblastomas
102. Coexpression of erythropoietin and vascular endothelial growth factor in nervous system tumors associated with von Hippel-Lindau tumor suppressor gene loss of function
103. VHL gene deletion and enhanced VEGF gene expression detected in the stromal cells of retinal angioma
104. Conditional switching of vascular endothelial growth factor (VEGF) expression in tumors: Induction of endothelial cell shedding and regression of hemangioblastoma-like vessels by VEGF withdrawal
105. Transforming growth factor alpha is a target for the von Hippel-Lindau tumor suppressor
106. Role of transforming growth factor-alpha in VHL-/- clear cell renal carcinoma cell proliferation: A possible mechanism coupling von Hippel-Lindau tumor suppressor inactivation and tumorigenesis
107. Lessons from hereditary colorectal cancer
108. Markedly increased amounts of messenger RNAs for vascular endothelial growth factor and placenta growth factor in renal cell carcinoma associated with angiogenesis
109. Frequent overexpression of vascular endothelial growth factor gene in human renal cell carcinoma
110. Vascular permeability factor (vascular endothelial growth factor) gene is expressed differentially in normal tissues, macrophages, and tumors
111. Oncogenic alterations of metabolism
112. Down-regulation of transmembrance carbonic anhydrases in renal cell carcinoma cell lines by wild-type von Hippel-Lindau transgenes
113. Differential response to transforming growth factor (TGF)-alpha and fibroblast growth factor (FGF) in human renal cell carcinomas of the clear cell and papillary types
114. Effects of transforming growth factor-beta, transforming growth factor-alpha, and other growth factors on renal proximal tubule cells
115. Modulation of pro-epidermal growth factor, pro-transforming growth factor alpha and epidermal growth factor receptor gene expression in human renal carcinomas
116. Growth regulation of human renal carcinoma cells: Role of transforming growth factor alpha
117. Suppression of growth of renal carcinoma cells by the von Hippel-Lindau tumor pressor gene
118. Von Hippel-Lindau gene-mediated growth suppression and induction of differentiation in renal cell carcinoma cells grown as multicellular tumor spheroids
119. The von Hippel-Lindau tumor suppressor gene is required for cell cycle exit upon serum withdrawal
120. VHL induces renal cell differentiation and growth arrest through integration of cell-cell and cell-extracellular matrix signaling