(Q28220414)

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Molecular basis of the VHL hereditary cancer syndrome

scientific article (publication date: September 2002)

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Europe PubMed Central

Molecular basis of the VHL hereditary cancer syndrome (English)

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William G. Kaelin

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William G Kaelin

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September 2002

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Nature Reviews Cancer

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2

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9

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673-82

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Über eine sehr seltene Erkrankung der Netzhaut

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

ZUR FRAGE DER ANGIOMATOSIS RETINae UND IHRER HIRNKOMPLIKATIONEN

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Haemangioblastoma of the central nervous system in von Hippel-Lindau disease. French VHL Study Group

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

von Hippel-Lindau disease

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Von Hippel-Lindau disease maps to the region of chromosome 3 associated with renal cell carcinoma

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Identification of the von Hippel-Lindau disease tumor suppressor gene

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Comparative sequence analysis of the VHL tumor suppressor gene

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

C. elegans EGL-9 and mammalian homologs define a family of dioxygenases that regulate HIF by prolyl hydroxylation

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

A conserved family of prolyl-4-hydroxylases that modify HIF

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Germline mutations in the von Hippel-Lindau disease tumor suppressor gene: correlations with phenotype

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Genotype-phenotype correlations in von Hippel-Lindau disease

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Germline and somatic mutations in von Hippel-Lindau disease gene and its significance in the development of kidney cancer

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Mutations of the VHL tumour suppressor gene in renal carcinoma

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

von Hippel-Lindau gene deletion detected in the stromal cell component of a cerebellar hemangioblastoma associated with von Hippel-Lindau disease

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

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

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Somatic mutations of the von Hippel-Lindau disease tumour suppressor gene in non-familial clear cell renal carcinoma

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Mutations of the VHL gene in sporadic renal cell carcinoma: definition of a risk factor for VHL patients to develop an RCC

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Silencing of the VHL tumor-suppressor gene by DNA methylation in renal carcinoma

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Germ-line mutations in nonsyndromic pheochromocytoma

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Genetic predisposition to phaeochromocytoma: analysis of candidate genes GDNF, RET and VHL.

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Sporadic phaeochromocytomas are rarely associated with germline mutations in the von Hippel-Lindau and RET genes

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Tumour suppression by the human von Hippel-Lindau gene product

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

pVHL19 is a biologically active product of the von Hippel-Lindau gene arising from internal translation initiation

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Alternate choice of initiation codon produces a biologically active product of the von Hippel Lindau gene with tumor suppressor activity

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

A second major native von Hippel-Lindau gene product, initiated from an internal translation start site, functions as a tumor suppressor.

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Immunostaining of the von Hippel-Lindau gene product in normal and neoplastic human tissues

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Transcription-dependent nuclear-cytoplasmic trafficking is required for the function of the von Hippel-Lindau tumor suppressor protein

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Ran-mediated nuclear export of the von Hippel-Lindau tumor suppressor protein occurs independently of its assembly with cullin-2

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Role of exon 2-encoded beta -domain of the von Hippel-Lindau tumor suppressor protein

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Characterization of the VHL tumor suppressor gene product: localization, complex formation, and the effect of natural inactivating mutations

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Inhibition of Transcription Elongation by the VHL Tumor Suppressor Protein

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The von Hippel-Lindau tumor-suppressor gene product forms a stable complex with human CUL-2, a member of the Cdc53 family of proteins

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

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

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Binding of the von Hippel-Lindau Tumor Suppressor Protein to Elongin B and C

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Regulation of hypoxia-inducible mRNAs by the von Hippel-Lindau tumor suppressor protein requires binding to complexes containing elongins B/C and Cul2

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

SKP1 connects cell cycle regulators to the ubiquitin proteolysis machinery through a novel motif, the F-box

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

SCF and Cullin/Ring H2-based ubiquitin ligases

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Structure of the VHL-ElonginC-ElonginB complex: implications for VHL tumor suppressor function

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The von Hippel-Lindau tumor suppressor protein is a component of an E3 ubiquitin-protein ligase activity

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Identification of the von Hippel-lindau tumor-suppressor protein as part of an active E3 ubiquitin ligase complex

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Rbx1, a component of the VHL tumor suppressor complex and SCF ubiquitin ligase

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Post-transcriptional regulation of vascular endothelial growth factor mRNA by the product of the VHL tumor suppressor gene

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Putative control of angiogenesis in hemangioblastomas by the von Hippel-Lindau tumor suppressor gene.

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Negative regulation of hypoxia-inducible genes by the von Hippel-Lindau protein

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Regulation of mammalian O2 homeostasis by hypoxia-inducible factor 1

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The tumour suppressor protein VHL targets hypoxia-inducible factors for oxygen-dependent proteolysis

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Hypoxia inducible factor-alpha binding and ubiquitylation by the von Hippel-Lindau tumor suppressor protein

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Ubiquitination of hypoxia-inducible factor requires direct binding to the beta-domain of the von Hippel-Lindau protein

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Activation of HIF1alpha ubiquitination by a reconstituted von Hippel-Lindau (VHL) tumor suppressor complex

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Mechanism of regulation of the hypoxia-inducible factor-1 alpha by the von Hippel-Lindau tumor suppressor protein

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

HIFalpha targeted for VHL-mediated destruction by proline hydroxylation: implications for O2 sensing

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Targeting of HIF-alpha to the von Hippel-Lindau ubiquitylation complex by O2-regulated prolyl hydroxylation

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

HIF-1alpha binding to VHL is regulated by stimulus-sensitive proline hydroxylation

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https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Structural basis for the recognition of hydroxyproline in HIF-1 alpha by pVHL

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Structure of an HIF-1alpha -pVHL complex: hydroxyproline recognition in signaling

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Structural and mechanistic studies on 2-oxoglutarate-dependent oxygenases and related enzymes

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Prolyl 4-hydroxylases and their protein disulfide isomerase subunit

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Characterization and comparative analysis of the EGLN gene family

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Transcription. Oxygen sensing gets a second wind

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Asparagine hydroxylation of the HIF transactivation domain a hypoxic switch

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Carboxyl-terminal transactivation activity of hypoxia-inducible factor 1 alpha is governed by a von Hippel-Lindau protein-independent, hydroxylation-regulated association with p300/CBP.

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Structural basis for recruitment of CBP/p300 by hypoxia-inducible factor-1 alpha

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Structural basis for Hif-1 alpha /CBP recognition in the cellular hypoxic response

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Hypoxia-inducible factor (HIF) asparagine hydroxylase is identical to factor inhibiting HIF (FIH) and is related to the cupin structural family

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Identification of novel hypoxia dependent and independent target genes of the von Hippel-Lindau (VHL) tumour suppressor by mRNA differential expression profiling

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Identification of alternative spliced variants of human hypoxia-inducible factor-1alpha

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

von Hippel-Lindau protein mutants linked to type 2C VHL disease preserve the ability to downregulate HIF.

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Induction of hypervascularity without leakage or inflammation in transgenic mice overexpressing hypoxia-inducible factor-1alpha

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Angiogenesis is induced in a rabbit model of hindlimb ischemia by naked DNA encoding an HIF-1alpha/VP16 hybrid transcription factor

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Direct Interaction of the β-Domain of VHL Tumor Suppressor Protein with the Regulatory Domain of Atypical PKC Isotypes

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The von Hippel-Lindau tumor suppressor protein mediates ubiquitination of activated atypical protein kinase C.

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

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

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Activation of Sp1-mediated vascular permeability factor/vascular endothelial growth factor transcription requires specific interaction with protein kinase C zeta

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

An important von Hippel-Lindau tumor suppressor domain mediates Sp1-binding and self-association

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The von Hippel-Lindau tumor suppressor gene product interacts with Sp1 to repress vascular endothelial growth factor promoter activity

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Formation of the VHL-elongin BC tumor suppressor complex is mediated by the chaperonin TRiC.

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Diverse effects of mutations in exon II of the von Hippel-Lindau (VHL) tumor suppressor gene on the interaction of pVHL with the cytosolic chaperonin and pVHL-dependent ubiquitin ligase activity

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The von Hippel-Lindau tumor suppressor protein is required for proper assembly of an extracellular fibronectin matrix

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Endoplasmic reticulum/cytosolic localization of von Hippel-Lindau gene products is mediated by a 64-amino acid region

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Protective function of von Hippel-Lindau protein against impaired protein processing in renal carcinoma cells

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The von Hippel-Lindau tumor suppressor gene inhibits hepatocyte growth factor/scatter factor-induced invasion and branching morphogenesis in renal carcinoma cells

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Expression of growth factors and growth factor receptors in capillary hemangioblastoma

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Up-regulation of vascular endothelial growth factor in stromal cells of hemangioblastomas is correlated with up-regulation of the transcription factor HRF/HIF-2alpha

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Retinal hemangioblastoma. A histologic, immunohistochemical, and ultrastructural evaluation

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Expression of Vascular Endothelial Growth Factor in Capillary Hemangioblastoma

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

VHL gene deletion and enhanced VEGF gene expression detected in the stromal cells of retinal angioma

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

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

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Conditional switching of VEGF provides new insights into adult neovascularization and pro-angiogenic therapy

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Exogenous vascular endothelial growth factor induces malformed and hyperfused vessels during embryonic neovascularization

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Vascular tumors in livers with targeted inactivation of the von Hippel-Lindau tumor suppressor.

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Comprehensive allelotyping of human renal cell carcinomas using microsatellite DNA probes

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Molecular genetic investigation of sporadic renal cell carcinoma: analysis of allele loss on chromosomes 3p, 5q, 11p, 17 and 22

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Duplication of an approximately 1.5 Mb DNA segment at chromosome 5q22 indicates the locus of a new tumour gene in nonpapillary renal cell carcinomas

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The von Hippel-Lindau tumor suppressor gene is required for cell cycle exit upon serum withdrawal

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

VHL induces renal cell differentiation and growth arrest through integration of cell-cell and cell-extracellular matrix signaling

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Inhibition of HIF is necessary for tumor suppression by the von Hippel-Lindau protein

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The contribution of VHL substrate binding and HIF1-alpha to the phenotype of VHL loss in renal cell carcinoma

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

The hypoxia-responsive transcription factor EPAS1 is essential for catecholamine homeostasis and protection against heart failure during embryonic development

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

von Hippel-Lindau Protein Induces Hypoxia-regulated Arrest of Tyrosine Hydroxylase Transcript Elongation in Pheochromocytoma Cells

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

E2F-1 functions in mice to promote apoptosis and suppress proliferation

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

HIF-1 alpha is required for solid tumor formation and embryonic vascularization.

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Hypoxia-inducible factor-1 modulates gene expression in solid tumors and influences both angiogenesis and tumor growth.

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Anticancer drug targets: approaching angiogenesis

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Mechanism-based target identification and drug discovery in cancer research

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Cooperative inhibition of renal cancer growth by anti-epidermal growth factor receptor antibody and protein kinase A antisense oligonucleotide

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Treatment of polycystic kidney disease with a novel tyrosine kinase inhibitor

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Paradoxical secondary polycythemia in von Hippel-Lindau patients treated with anti-vascular endothelial growth factor receptor therapy

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Protein tyrosine kinase inhibitors: new treatment modalities?

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

Inhibition of collagen synthesis with prolyl 4-hydroxylase inhibitor improves left ventricular function and alters the pattern of left ventricular dilatation after myocardial infarction

1 reference

https://api.crossref.org/works/10.1038%2FNRC885

31 March 2020

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