VHL, the story of a tumour suppressor gene

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VHL, the story of a tumour suppressor gene is …
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P6179Dimensions Publication ID1009041579
P356DOI10.1038/NRC3844
P698PubMed publication ID25533676

P50authorEamonn R MaherQ56435841
P2093author name stringTim Eisen
Lucy Gossage
P2860cites workStructure of the VHL-ElonginC-ElonginB complex: implications for VHL tumor suppressor functionQ22009393
The tumour suppressor protein VHL targets hypoxia-inducible factors for oxygen-dependent proteolysisQ22009936
HIFalpha targeted for VHL-mediated destruction by proline hydroxylation: implications for O2 sensingQ24291102
A conserved family of prolyl-4-hydroxylases that modify HIFQ24291794
Structure of an HIF-1alpha -pVHL complex: hydroxyproline recognition in signalingQ24297062
Structural basis for the recognition of hydroxyproline in HIF-1 alpha by pVHLQ24299061
Inhibition of Transcription Elongation by the VHL Tumor Suppressor ProteinQ24336629
Binding of the von Hippel-Lindau Tumor Suppressor Protein to Elongin B and CQ24336712
Exome sequencing identifies frequent mutation of the SWI/SNF complex gene PBRM1 in renal carcinomaQ24337664
Mutation of the Von Hippel-Lindau tumour suppressor gene in capillary haemangioblastomas of the central nervous systemQ71398598
[Results of mutation analyses of von Hippel-Lindau disease gene in Japanese patients: comparison with results in United States and United Kingdom]Q71549928
Markedly increased amounts of messenger RNAs for vascular endothelial growth factor and placenta growth factor in renal cell carcinoma associated with angiogenesisQ71634131
Up-regulation of vascular endothelial growth factor and its receptors in von Hippel-Lindau disease-associated and sporadic hemangioblastomasQ71678927
Cellular proteins that bind the von Hippel-Lindau disease gene product: mapping of binding domains and the effect of missense mutationsQ71729095
Frequent overexpression of vascular endothelial growth factor gene in human renal cell carcinomaQ72522741
Molecular genetic investigations of the mechanism of tumourigenesis in von Hippel-Lindau disease: analysis of allele loss in VHL tumoursQ72679743
Analysis of multiple renal cell adenomas and carcinomas suggests allelic loss at 3p21 to be a prerequisite for malignant developmentQ73590491
Transforming growth factor alpha is a target for the von Hippel-Lindau tumor suppressorQ74093367
Inhibitors of Transcription, Proteasome Inhibitors, and DNA-Damaging Drugs Differentially Affect Feedback of p53 DegradationQ77403566
von Hippel-Lindau tumor suppressor protein represses platelet-derived growth factor B-chain gene expression via the Sp1 binding element in the proximal PDGF-B promoterQ77986081
The von Hippel-Lindau tumor suppressor protein sensitizes renal cell carcinoma cells to tumor necrosis factor-induced cytotoxicity by suppressing the nuclear factor-kappaB-dependent antiapoptotic pathwayQ79286125
Collagen matrix assembly is driven by the interaction of von Hippel-Lindau tumor suppressor protein with hydroxylated collagen IV alpha 2Q80799407
Role of elongin-binding domain of von Hippel Lindau gene product on HuR-mediated VPF/VEGF mRNA stability in renal cell carcinomaQ81232141
Folding and quality control of the VHL tumor suppressor proceed through distinct chaperone pathwaysQ81813867
Von hippel-lindau disease and erythrocytosisQ83578987
Genotype-phenotype correlations in VHL exon deletionsQ84571474
Interplay between HDAC3 and WDR5 is essential for hypoxia-induced epithelial-mesenchymal transitionQ84872949
Regulation of Hypoxia-Inducible mRNAs by the von Hippel-Lindau Tumor Suppressor Protein Requires Binding to Complexes Containing Elongins B/C and Cul2Q24523680
von Hippel-Lindau protein binds hyperphosphorylated large subunit of RNA polymerase II through a proline hydroxylation motif and targets it for ubiquitinationQ24554216
HIF-1alpha binding to VHL is regulated by stimulus-sensitive proline hydroxylationQ24555773
The von Hippel-Lindau tumor-suppressor gene product forms a stable complex with human CUL-2, a member of the Cdc53 family of proteinsQ24564802
Mechanism of regulation of the hypoxia-inducible factor-1 alpha by the von Hippel-Lindau tumor suppressor proteinQ24599442
Mutation and cancer: statistical study of retinoblastomaQ24618185
von Hippel-Lindau disease: a clinical and scientific reviewQ24622030
The Catalogue of Somatic Mutations in Cancer (COSMIC)Q24653244
Identification of the von Hippel-lindau tumor-suppressor protein as part of an active E3 ubiquitin ligase complexQ24654714
pVHL acts as an adaptor to promote the inhibitory phosphorylation of the NF-kappaB agonist Card9 by CK2Q24684757
Inhibition of HIF2alpha is sufficient to suppress pVHL-defective tumor growthQ24800550
Genetic analysis of pathways regulated by the von Hippel-Lindau tumor suppressor in Caenorhabditis elegansQ24804603
HIF1α and HIF2α: sibling rivalry in hypoxic tumour growth and progressionQ27025886
Artificial ligand binding within the HIF2  PAS-B domain of the HIF2 transcription factorQ27653363
Allosteric inhibition of hypoxia inducible factor-2 with small moleculesQ27676596
Adverse outcomes in clear cell renal cell carcinoma with mutations of 3p21 epigenetic regulators BAP1 and SETD2: a report by MSKCC and the KIRC TCGA research networkQ27852242
Comprehensive molecular characterization of clear cell renal cell carcinomaQ27852374
Integrated molecular analysis of clear-cell renal cell carcinomaQ27852382
The role of aberrant VHL/HIF pathway elements in predicting clinical outcome to pazopanib therapy in patients with metastatic clear-cell renal cell carcinoma.Q27852466
Targeting of HIF-alpha to the von Hippel-Lindau ubiquitylation complex by O2-regulated prolyl hydroxylationQ27860876
Intratumor Heterogeneity and Branched Evolution Revealed by Multiregion SequencingQ27860948
Clustering of features of von Hippel-Lindau syndrome: evidence for a complex genetic locusQ28242901
Formation of primary cilia in the renal epithelium is regulated by the von Hippel-Lindau tumor suppressor proteinQ28246139
Neuronal apoptosis linked to EglN3 prolyl hydroxylase and familial pheochromocytoma genes: developmental culling and cancerQ28267037
The concept of synthetic lethality in the context of anticancer therapyQ28268061
Rbx1, a component of the VHL tumor suppressor complex and SCF ubiquitin ligaseQ28506124
pVHL function is essential for endothelial extracellular matrix deposition.Q28589604
HIF-2alpha regulates murine hematopoietic development in an erythropoietin-dependent mannerQ28589605
Inactivation of the arylhydrocarbon receptor nuclear translocator (Arnt) suppresses von Hippel-Lindau disease-associated vascular tumors in miceQ28590550
Acute postnatal ablation of Hif-2alpha results in anemiaQ28591898
Hypoxia-inducible factor-2 (HIF-2) regulates hepatic erythropoietin in vivoQ28594830
Binding of natively unfolded HIF-1alpha ODD domain to p53Q28910406
Systematic sequencing of renal carcinoma reveals inactivation of histone modifying genesQ29615757
Ubiquitination of hypoxia-inducible factor requires direct binding to the beta-domain of the von Hippel-Lindau proteinQ29615928
Hypoxia inducible factor-alpha binding and ubiquitylation by the von Hippel-Lindau tumor suppressor proteinQ29615931
High-resolution genome-wide mapping of HIF-binding sites by ChIP-seq.Q36029877
von Hippel-Lindau mutation in mice recapitulates Chuvash polycythemia via hypoxia-inducible factor-2alpha signaling and splenic erythropoiesisQ36121776
A rationally designed small molecule that inhibits the HIF-1alpha-ARNT heterodimer from binding to DNA in vivoQ36130851
Hypoxia-inducible factors: mediators of cancer progression and targets for cancer therapyQ36220497
A second major native von Hippel-Lindau gene product, initiated from an internal translation start site, functions as a tumor suppressor.Q36222472
Renal cyst development in mice with conditional inactivation of the von Hippel-Lindau tumor suppressorQ36446896
The von Hippel-Lindau tumor suppressor protein and Egl-9-Type proline hydroxylases regulate the large subunit of RNA polymerase II in response to oxidative stressQ36540303
Influence of the RNA-binding protein HuR in pVHL-regulated p53 expression in renal carcinoma cellsQ36549605
The positive regulation of p53 by the tumor suppressor VHL.Q36592019
Molecular pathways in renal cell carcinoma--rationale for targeted treatmentQ36625166
Clinical and pathologic impact of select chromatin-modulating tumor suppressors in clear cell renal cell carcinomaQ36736210
The phenotype of polycythemia due to Croatian homozygous VHL (571C>G:H191D) mutation is different from that of Chuvash polycythemia (VHL 598C>T:R200W)Q36862486
Targeting GLUT1 and the Warburg Effect in Renal Cell Carcinoma by Chemical Synthetic LethalityQ36930538
Hypoxia-inducible factor-2 regulates vascular tumorigenesis in miceQ36953894
Kinase requirements in human cells: III. Altered kinase requirements in VHL-/- cancer cells detected in a pilot synthetic lethal screenQ36954859
Downregulation of integrins by von Hippel-Lindau (VHL) tumor suppressor protein is independent of VHL-directed hypoxia-inducible factor alpha degradationQ37001497
HIF-alpha effects on c-Myc distinguish two subtypes of sporadic VHL-deficient clear cell renal carcinomaQ37052897
Integrative analysis of HIF binding and transactivation reveals its role in maintaining histone methylation homeostasisQ37132758
Elongin BC complex prevents degradation of von Hippel-Lindau tumor suppressor gene productsQ37230912
pVHL modification by NEDD8 is required for fibronectin matrix assembly and suppression of tumor developmentQ37275211
Post-transcriptional regulation of vascular endothelial growth factor mRNA by the product of the VHL tumor suppressor geneQ37285776
Negative regulation of hypoxia-inducible genes by the von Hippel-Lindau proteinQ37286026
Hypoxia-inducible factor 2 regulates hepatic lipid metabolismQ37302297
Emerging roles of microRNAs in the molecular responses to hypoxiaQ37635252
Novel homozygous VHL mutation in exon 2 is associated with congenital polycythemia but not with cancer.Q37685596
Alterations in VHL as potential biomarkers in renal-cell carcinomaQ37724576
Oxygen sensing, homeostasis, and diseaseQ37914865
Treatment selection in metastatic renal cell carcinoma: expert consensusQ37999655
Generation of a Mouse Model of Von Hippel–Lindau Kidney Disease Leading to Renal Cancers by Expression of a Constitutively Active Mutant of HIF1αQ39042077
The von Hippel-Lindau Chuvash mutation promotes pulmonary hypertension and fibrosis in mice.Q39250750
HIF2α acts as an mTORC1 activator through the amino acid carrier SLC7A5.Q39252741
Loss of JAK2 regulation via a heterodimeric VHL-SOCS1 E3 ubiquitin ligase underlies Chuvash polycythemia.Q39273902
The von Hippel-Lindau tumor suppressor protein regulates gene expression and tumor growth through histone demethylase JARID1C.Q39513689
VHL Gene Mutations and Their Effects on Hypoxia Inducible Factor HIFα: Identification of Potential Driver and Passenger MutationsQ39515837
Priming-dependent phosphorylation and regulation of the tumor suppressor pVHL by glycogen synthase kinase 3.Q34569182
Identification and evaluation of soft coral diterpenes as inhibitors of HIF-2α induced gene expression.Q34700627
Von Hippel-Lindau disease in a Newfoundland kindredQ34726572
The von Hippel–Lindau tumor suppressor gene is required for cell cycle exit upon serum withdrawalQ34744818
The von Hippel-Lindau Tumor Suppressor Protein Is Required for Proper Assembly of an Extracellular Fibronectin MatrixQ34748818
pVHL 19 is a biologically active product of the von Hippel–Lindau gene arising from internal translation initiationQ34753344
Quantitative image analysis identifies pVHL as a key regulator of microtubule dynamic instabilityQ35005618
Acriflavine inhibits HIF-1 dimerization, tumor growth, and vascularizationQ35006577
Failure to prolyl hydroxylate hypoxia-inducible factor alpha phenocopies VHL inactivation in vivoQ35065823
A new hypoxia inducible factor-2 inhibitory pyrrolinone alkaloid from roots and stems of Piper sarmentosum.Q35194070
The von Hippel-Lindau tumor suppressor protein is a component of an E3 ubiquitin-protein ligase activityQ35201819
Genetic and Functional Studies Implicate HIF1α as a 14q Kidney Cancer Suppressor GeneQ35452783
Reconsideration of biallelic inactivation of the VHL tumour suppressor gene in hemangioblastomas of the central nervous systemQ35462032
Tumor cell invasion of von Hippel Lindau renal cell carcinoma cells is mediated by membrane type-1 matrix metalloproteinaseQ35574224
VHL promotes E2 box-dependent E-cadherin transcription by HIF-mediated regulation of SIP1 and snailQ35641669
Identification of membrane type-1 matrix metalloproteinase as a target of hypoxia-inducible factor-2 alpha in von Hippel-Lindau renal cell carcinomaQ35741707
Germ-line mutations in the von Hippel-Lindau tumor-suppressor gene are similar to somatic von Hippel-Lindau aberrations in sporadic renal cell carcinoma.Q35889809
HIF-1 antagonizes p53-mediated apoptosis through a secreted neuronal tyrosinaseQ35893950
Histone methyltransferase gene SETD2 is a novel tumor suppressor gene in clear cell renal cell carcinoma.Q39700386
Software and database for the analysis of mutations in the VHL geneQ39722266
NF-kappaB-dependent plasticity of the epithelial to mesenchymal transition induced by Von Hippel-Lindau inactivation in renal cell carcinomasQ39752810
Suppression of hypoxia-inducible factor 2alpha restores p53 activity via Hdm2 and reverses chemoresistance of renal carcinoma cellsQ39773550
VHL loss causes spindle misorientation and chromosome instability.Q39822734
Regulation of renal epithelial tight junctions by the von Hippel-Lindau tumor suppressor gene involves occludin and claudin 1 and is independent of E-cadherinQ39906454
von Hippel-Lindau tumor suppressor gene-dependent mRNA stabilization of the survival factor parathyroid hormone-related protein in human renal cell carcinoma by the RNA-binding protein HuR.Q39908665
Regulation of Jumonji-domain-containing histone demethylases by hypoxia-inducible factor (HIF)-1alphaQ39949245
The von Hippel-Lindau Tumor Suppressor Gene Product Interacts with Sp1 To Repress Vascular Endothelial Growth Factor Promoter ActivityQ40023447
Hypoxia-inducible factor linked to differential kidney cancer risk seen with type 2A and type 2B VHL mutations.Q40127751
Interaction of hydroxylated collagen IV with the von hippel-lindau tumor suppressor.Q40163119
Loss of the actin regulator HSPC300 results in clear cell renal cell carcinoma protection in Von Hippel-Lindau patientsQ40169023
Development of a cell-based reporter assay for screening of inhibitors of hypoxia-inducible factor 2-induced gene expressionQ40252871
Primary cilium formation requires von hippel-lindau gene function in renal-derived cellsQ40254089
Role of hypoxia-inducible factor (HIF)-1alpha versus HIF-2alpha in the regulation of HIF target genes in response to hypoxia, insulin-like growth factor-I, or loss of von Hippel-Lindau function: implications for targeting the HIF pathwayQ40266970
Activation of HIF2α in Kidney Proximal Tubule Cells Causes Abnormal Glycogen Deposition but not TumorigenesisQ40321378
Characterization of a von Hippel Lindau pathway involved in extracellular matrix remodeling, cell invasion, and angiogenesisQ40322124
Stromal cell-derived factor-1alpha and CXCR4 expression in hemangioblastoma and clear cell-renal cell carcinoma: von Hippel-Lindau loss-of-function induces expression of a ligand and its receptorQ40396846
VHL expression in renal cell carcinoma sensitizes to bortezomib (PS-341) through an NF-kappaB-dependent mechanism.Q40479170
Inhibition of hypoxia-inducible factor is sufficient for growth suppression of VHL-/- tumors.Q40585447
Chemokine receptor CXCR4 downregulated by von Hippel-Lindau tumour suppressor pVHL.Q40633683
Gene expression profiling in a renal cell carcinoma cell line: dissecting VHL and hypoxia-dependent pathwaysQ40655882
An important von Hippel-Lindau tumor suppressor domain mediates Sp1-binding and self-associationQ40914690
Tumor cells are the site of erythropoietin synthesis in human renal cancers associated with polycythemiaQ41222952
Tumour suppression by the human von Hippel-Lindau gene productQ41320477
Modulating hypoxia-inducible transcription by disrupting the HIF-1-DNA interfaceQ41857906
Analysis of VHL Gene Alterations and their Relationship to Clinical Parameters in Sporadic Conventional Renal Cell CarcinomaQ41919666
Renal oxygenation suppresses VHL loss-induced senescence that is caused by increased sensitivity to oxidative stressQ42413615
Alternate choice of initiation codon produces a biologically active product of the von Hippel Lindau gene with tumor suppressor activityQ42469993
Genetic basis of congenital erythrocytosis: mutation update and online databasesQ42622691
Mutations of the VHL tumour suppressor gene in renal carcinomaQ42806703
Biomarkers predicting outcome in patients with advanced renal cell carcinoma: Results from sorafenib phase III Treatment Approaches in Renal Cancer Global Evaluation TrialQ42962024
The histone demethylases JMJD1A and JMJD2B are transcriptional targets of hypoxia-inducible factor HIF.Q43154570
Genetic deletion of the long isoform of the von Hippel-Lindau tumour suppressor gene product alters microtubule dynamicsQ43426283
VHL loss actuates a HIF-independent senescence programme mediated by Rb and p400.Q43455466
Clinical and pathological impact of VHL, PBRM1, BAP1, SETD2, KDM6A, and JARID1c in clear cell renal cell carcinoma.Q43806776
Role of the von Hippel-Lindau tumor suppressor gene in the formation of beta1-integrin fibrillar adhesionsQ43999004
Inhibition of HIF is necessary for tumor suppression by the von Hippel-Lindau proteinQ44043145
The contribution of VHL substrate binding and HIF1-alpha to the phenotype of VHL loss in renal cell carcinomaQ44043146
HIF activation identifies early lesions in VHL kidneys: evidence for site-specific tumor suppressor function in the nephronQ44068443
Mutations in the VHL gene in sporadic apparently congenital polycythemiaQ44189671
Genotype-phenotype correlation in von Hippel-Lindau families with renal lesions.Q44230145
Prospective study assessing hypoxia-related proteins as markers for the outcome of treatment with sunitinib in advanced clear-cell renal cell carcinoma.Q44247719
Loss of von Hippel-Lindau protein causes cell density dependent deregulation of CyclinD1 expression through hypoxia-inducible factor.Q44790615
Germline mutations in the von Hippel-Lindau disease tumor suppressor gene: correlations with phenotypeQ44847029
Targeted exome sequencing in clear cell renal cell carcinoma tumors suggests aberrant chromatin regulation as a crucial step in ccRCC development.Q45880509
Molecular study of congenital erythrocytosis in 70 unrelated patients revealed a potential causal mutation in less than half of the cases (Where is/are the missing gene(s)?).Q45897769
Exploring the limits of benzimidazole DNA-binding oligomers for the hypoxia inducible factor (HIF) site.Q45981766
Clinical response to therapy targeted at vascular endothelial growth factor in metastatic renal cell carcinoma: impact of patient characteristics and Von Hippel-Lindau gene status.Q46014175
Zebrafish mutants in the von Hippel-Lindau tumor suppressor display a hypoxic response and recapitulate key aspects of Chuvash polycythemiaQ46079205
Alu-Alu recombination underlies the vast majority of large VHL germline deletions: Molecular characterization and genotype-phenotype correlations in VHL patientsQ46091220
von Hippel-Lindau gene status and response to vascular endothelial growth factor targeted therapy for metastatic clear cell renal cell carcinomaQ46478024
Potential histologic and molecular predictors of response to temsirolimus in patients with advanced renal cell carcinomaQ46876961
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 tumourigenesisQ47981361
Fibronectin is a hypoxia-independent target of the tumor suppressor VHL.Q48035055
Coexpression of erythropoietin and vascular endothelial growth factor in nervous system tumors associated with von Hippel-Lindau tumor suppressor gene loss of functionQ48367527
Renal cell carcinoma risk in type 2 von Hippel-Lindau disease correlates with defects in pVHL stability and HIF-1alpha interactions.Q53352910
The VHL tumor suppressor: riding tandem with GSK3beta in primary cilium maintenanceQ53536706
Somatic VHL alteration and its impact on prognosis in patients with clear cell renal cell carcinoma.Q53623968
Über eine sehr seltene Erkrankung der NetzhautQ56068022
ZUR FRAGE DER ANGIOMATOSIS RETINae UND IHRER HIRNKOMPLIKATIONENQ56068023
Frequent mutations of genes encoding ubiquitin-mediated proteolysis pathway components in clear cell renal cell carcinomaQ57177515
Direct Interaction of the β-Domain of VHL Tumor Suppressor Protein with the Regulatory Domain of Atypical PKC IsotypesQ58323654
Loss of alleles of loci on the short arm of chromosome 3 in renal cell carcinomaQ59077568
Specific genetic change in tumors associated with von Hippel-Lindau diseaseQ69641054
Identification of the von Hippel-Lindau disease tumor suppressor geneQ29618644
Differential roles of hypoxia-inducible factor 1alpha (HIF-1alpha) and HIF-2alpha in hypoxic gene regulationQ29619687
Mutations of the VHL gene in sporadic renal cell carcinoma: definition of a risk factor for VHL patients to develop an RCCQ29937783
Genotype-phenotype correlations in von Hippel-Lindau disease.Q30357251
Whole-exome sequencing of human pancreatic cancers and characterization of genomic instability caused by MLH1 haploinsufficiency and complete deficiencyQ30465158
The von Hippel-Lindau tumor suppressor protein controls ciliogenesis by orienting microtubule growthQ30480543
Small-molecule inhibitors of HIF-2a translation link its 5'UTR iron-responsive element to oxygen sensingQ33396143
Analysis of germline variants in CDH1, IGFBP3, MMP1, MMP3, STK15 and VEGF in familial and sporadic renal cell carcinomaQ33473894
Statistical analysis of the two stage mutation model in von Hippel-Lindau disease, and in sporadic cerebellar haemangioblastoma and renal cell carcinomaQ33597694
Von Hippel-Lindau (VHL) disease: distinct phenotypes suggest more than one mutant allele at the VHL locusQ33641198
A molecule targeting VHL-deficient renal cell carcinoma that induces autophagyQ33642615
Mutations in the RET proto-oncogene and the von Hippel-Lindau disease tumour suppressor gene in sporadic and syndromic phaeochromocytomasQ33682913
Genetic and pharmacological strategies to refunctionalize the von Hippel Lindau R167Q mutant proteinQ33719533
Molecular genetics of clear-cell renal cell carcinomaQ33729097
Contrasting properties of hypoxia-inducible factor 1 (HIF-1) and HIF-2 in von Hippel-Lindau-associated renal cell carcinoma.Q33863055
Somatic mutations of the histone H3K27 demethylase gene UTX in human cancerQ33865946
Formation of the VHL-elongin BC tumor suppressor complex is mediated by the chaperonin TRiC.Q33886538
Function in protein folding of TRiC, a cytosolic ring complex containing TCP-1 and structurally related subunitsQ33938572
Contrasting effects on HIF-1alpha regulation by disease-causing pVHL mutations correlate with patterns of tumourigenesis in von Hippel-Lindau disease.Q33945016
Genetic analysis of von Hippel-Lindau disease.Q34098432
Disruption of oxygen homeostasis underlies congenital Chuvash polycythemiaQ34157665
Agents That Stabilize Mutated von Hippel–Lindau (VHL) Protein: Results of a High-Throughput Screen to Identify Compounds That Modulate VHL ProteostasisQ34167307
BAP1 loss defines a new class of renal cell carcinomaQ34280223
Solid renal tumor severity in von Hippel Lindau disease is related to germline deletion length and location.Q34286240
Somatic mutations of the von Hippel-Lindau disease tumour suppressor gene in non-familial clear cell renal carcinomaQ34320299
An Analysis of Phenotypic Variation in the Familial Cancer Syndrome von Hippel–Lindau Disease: Evidence for Modifier EffectsQ34387297
Lack of a Functional VHL Gene Product Sensitizes Renal Cell Carcinoma Cells to the Apoptotic Effects of the Protein Synthesis Inhibitor Verrucarin AQ34404515
von Hippel-Lindau protein mutants linked to type 2C VHL disease preserve the ability to downregulate HIF.Q34514928
Germ-line mutations in nonsyndromic pheochromocytomaQ34523021
VHL-mediated hypoxia regulation of cyclin D1 in renal carcinoma cells.Q34523542
Identification of cyclin D1 and other novel targets for the von Hippel-Lindau tumor suppressor gene by expression array analysis and investigation of cyclin D1 genotype as a modifier in von Hippel-Lindau disease.Q34524947
Regulation of microtubule stability by the von Hippel-Lindau tumour suppressor protein pVHL.Q34530199
Hypoxia inducible factor activates the transforming growth factor-alpha/epidermal growth factor receptor growth stimulatory pathway in VHL(-/-) renal cell carcinoma cellsQ34536583
LINDAU'S DISEASE. REVIEW OF THE LITERATURE AND STUDY OF A LARGE KINDRED.Q34540867
p53 stabilization and transactivation by a von Hippel-Lindau proteinQ34567288
P433issue1
P407language of work or nameEnglishQ1860
P304page(s)55-64
P577publication date2015-01-01
P13046publication type of scholarly workreview articleQ7318358
P1433published inNature Reviews CancerQ641657
P1476titleVHL, the story of a tumour suppressor gene
P478volume15

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Q39292822Genetic kidney diseases: Caenorhabditis elegans as model system
Q45047268Genetics of Von Hippel-Lindau Disease.
Q52622727Genotype and phenotype correlation in von Hippel-Lindau disease based on alteration of the HIF-α binding site in VHL protein
Q47362904Genotype phenotype correlation in Asian Indian von Hippel-Lindau (VHL) syndrome patients with pheochromocytoma/paraganglioma
Q36083146Genotype-phenotype analysis of von Hippel-Lindau syndrome in Korean families: HIF-α binding site missense mutations elevate age-specific risk for CNS hemangioblastoma
Q64081971Genotype-phenotype relations of the von Hippel-Lindau tumor suppressor inferred from a large-scale analysis of disease mutations and interactors
Q50227566Germline ESR2 mutation predisposes to medullary thyroid carcinoma and causes up-regulation of RET expression
Q92257354Glutathione Metabolism in Renal Cell Carcinoma Progression and Implications for Therapies
Q36099567HDAC 1 and 6 modulate cell invasion and migration in clear cell renal cell carcinoma
Q38431622HIF prolyl hydroxylase PHD3 regulates translational machinery and glucose metabolism in clear cell renal cell carcinoma
Q38666170HIF prolyl hydroxylase inhibitors for the treatment of renal anaemia and beyond
Q100464172HIF-2α is indispensable for regulatory T cell function
Q55083786Hereditary Renal Tumor Syndromes: Update on Diagnosis and Management.
Q52572027Hereditary renal cell carcinoma syndromes: diagnosis, surveillance and management.
Q53312284Hereditary tumor syndromes in neuropathology
Q42217007Homo-PROTACs: bivalent small-molecule dimerizers of the VHL E3 ubiquitin ligase to induce self-degradation.
Q26786759Hyperplasia in glands with hormone excess
Q55419613Hypoxia-Inducible Factor 2-Dependent Pathways Driving Von Hippel-Lindau-Deficient Renal Cancer.
Q36342313Hypoxia-inducible factor-2α stabilizes the von Hippel-Lindau (VHL) disease suppressor, Myb-related protein 2
Q40462478Identification and validation of an eight-gene expression signature for predicting high Fuhrman grade renal cell carcinoma.
Q59129654Immunogenomic Landscape Contributes to Hyperprogressive Disease after Anti-PD-1 Immunotherapy for Cancer
Q50159013In-silico VHL Gene Mutation Analysis and Prognosis of Pancreatic Neuroendocrine Tumors in von Hippel-Lindau Disease.
Q36161809Inactivation of the tumor suppressor gene von Hippel-Lindau (VHL) in granulocytes contributes to development of liver hemangiomas in a mouse model
Q101407728Increased chromosomal instability characterizes metastatic renal cell carcinoma
Q39395931Independent association of PD-L1 expression with noninactivated VHL clear cell renal cell carcinoma-A finding with therapeutic potential
Q53435219Integrated genomic analysis identifies clinically relevant subtypes of renal clear cell carcinoma
Q64093013Intra-Familial Phenotypic Heterogeneity and Telomere Abnormality in von Hippel- Lindau Disease: Implications for Personalized Surveillance Plan and Pathogenesis of VHL-Associated Tumors
Q91876542Investigation of the role of VHL-HIF signaling in DNA repair and apoptosis in zebrafish
Q41247181Isocitrate dehydrogenases in physiology and cancer: biochemical and molecular insight
Q38889283Key considerations in the treatment of von Hippel-Lindau disease
Q42086368Link between dysregulated hypoxia signaling and aberrant methylation in clear cell renal cell carcinoma?
Q38819629Long-term follow-up and clinical course of a rare case of von Hippel-Lindau disease: A case report and review of the literature
Q91874588Loss of ELF5-FBXW7 stabilizes IFNGR1 to promote the growth and metastasis of triple-negative breast cancer through interferon-γ signalling
Q47117026Loss of PBRM1 rescues VHL dependent replication stress to promote renal carcinogenesis
Q30275713Loss of Quiescence in von Hippel-Lindau Hemangioblastomas is Associated with Erythropoietin Signaling
Q39229171Mammalian SWI/SNF complexes in cancer: emerging therapeutic opportunities
Q37217474Mechanistically distinct cancer-associated mTOR activation clusters predict sensitivity to rapamycin.
Q28073174Medical treatment of renal cancer: new horizons
Q91791256Meloxicam, a Selective COX-2 Inhibitor, Mediates Hypoxia-Inducible Factor- (HIF-) 1α Signaling in Hepatocellular Carcinoma
Q39340415Metabolic Alterations in Cancer and Their Potential as Therapeutic Targets
Q64097631Metabolic profiling of cancer cells reveals genome-wide crosstalk between transcriptional regulators and metabolism
Q38698512Metastatic renal cell carcinoma without evidence of a renal primary
Q51158837MicroRNAs in clear cell renal cell carcinoma: biological functions and applications
Q59791159Modeling epigenetic modifications in renal development and disease with organoids and genome editing
Q38697094Multiple renal cancer susceptibility polymorphisms modulate the HIF pathway.
Q90006920Muscleblind-like 2 controls the hypoxia response of cancer cells
Q59793924Mutated Von Hippel-Lindau-renal cell carcinoma (RCC) promotes patients specific natural killer (NK) cytotoxicity
Q58573422Never-in-mitosis A-related kinase 8, a novel target of von-Hippel-Lindau tumor suppressor protein, promotes gastric cancer cell proliferation
Q90139170New Insights Into Pheochromocytoma Surveillance of Young Patients With VHL Missense Mutations
Q58103553Non-Coding Micro RNAs and Hypoxia-Inducible Factors Are Selenium Targets for Development of a Mechanism-Based Combination Strategy in Clear-Cell Renal Cell Carcinoma-Bench-to-Bedside Therapy
Q91863036Novel gene mutation in von Hippel-Lindau disease - a report of two cases
Q55516484Novel genotype-phenotype correlations in five Chinese families with Von Hippel-Lindau disease.
Q57822415Oncometabolite induced primary cilia loss in pheochromocytoma
Q52686884Overexpression of COPS3 promotes clear cell renal cell carcinoma progression via regulation of Phospho-AKT(Thr308), Cyclin D1 and Caspase-3.
Q90404451Oxygen Tension and the VHL-Hif1α Pathway Determine Onset of Neuronal Polarization and Cerebellar Germinal Zone Exit
Q35995926PBRM1 Regulates the Expression of Genes Involved in Metabolism and Cell Adhesion in Renal Clear Cell Carcinoma
Q47370222PBRM1 and VHL expression correlate in human clear cell renal cell carcinoma with differential association with patient's overall survival
Q38805814Parkin modulates expression of HIF-1α and HIF-3α during hypoxia in gliobastoma-derived cell lines in vitro
Q98647386Pathogenic germline variants in patients with features of hereditary renal cell carcinoma: evidence for further locus heterogeneity
Q38542150Pheochromocytoma: Gasping for Air.
Q47115530Phosphorylation and Ubiquitination Regulate Protein Phosphatase 5 Activity and Its Prosurvival Role in Kidney Cancer
Q38786652Phosphorylation-dependent cleavage regulates von Hippel Lindau proteostasis and function
Q54977962Pilot study of dovitinib in patients with von Hippel-Lindau disease.
Q28822022Potent and selective chemical probe of hypoxic signalling downstream of HIF-α hydroxylation via VHL inhibition
Q38970282Precision medicine from the renal cancer genome
Q39095254Prognostic and predictive value of VHL gene alteration in renal cell carcinoma: a meta-analysis and review
Q91574563Prognostic significance of VHL, HIF1A, HIF2A, VEGFA and p53 expression in patients with clear‑cell renal cell carcinoma treated with sunitinib as first‑line treatment
Q47097655Proteome profiling of clear cell renal cell carcinoma in von Hippel-Lindau patients highlights upregulation of Xaa-Pro aminopeptidase-1, an anti-proliferative and anti-migratory exoprotease.
Q36003500Proteotranscriptomic Analysis Reveals Stage Specific Changes in the Molecular Landscape of Clear-Cell Renal Cell Carcinoma
Q33292271RalBP1 and p19-VHL play an oncogenic role, and p30-VHL plays a tumor suppressor role during the blebbishield emergency program
Q90716279Rb1/Rbl1/Vhl loss induces mouse subretinal angiomatous proliferation and hemangioblastoma
Q37598376Reduction of hepatic fibrosis by overexpression of von Hippel-Lindau protein in experimental models of chronic liver disease
Q38404984Regulation of hypoxia responses by flavin adenine dinucleotide-dependent modulation of HIF-1α protein stability
Q50941188Renal cancer.
Q35610555Repression of hypoxia-inducible factor α signaling by Set7-mediated methylation
Q89461828Retinal haemangioblastomas in von Hippel-Lindau germline mutation carriers: progression, complications and treatment outcome
Q132042932Ribosomal Protein S6: A Potential Therapeutic Target against Cancer?
Q37701667Role of VHL, HIF1A and SDH on the expression of miR-210: Implications for tumoral pseudo-hypoxic fate.
Q37697020SENP1 promotes proliferation of clear cell renal cell carcinoma through activation of glycolysis
Q60921421SENP1-mediated deSUMOylation of USP28 regulated HIF-1α accumulation and activation during hypoxia response
Q38824719Safety of available treatment options for renal cell carcinoma
Q26744089Sequential pathogenesis of metastatic VHL mutant clear cell renal cell carcinoma: putting it together with a translational perspective
Q92969786Sex specific associations in genome wide association analysis of renal cell carcinoma
Q38646901Shorter telomere length increases age-related tumor risks in von Hippel-Lindau disease patients.
Q42434480Signs and genetics of rare cancer syndromes with gastroenterological features
Q55023971Single-cell genetic analysis validates cytopathological identification of circulating cancer cells in patients with clear cell renal cell carcinoma.
Q47884990Specific Localization of Missense Mutations in the VHL Gene in Clear Cell Renal Cell Carcinoma
Q38828242Structure, function and regulation of jade family PHD finger 1 (JADE1).
Q92372125Systematic Review: Perioperative Systemic Therapy for Metastatic Renal Cell Carcinoma
Q99608172TET is targeted for proteasomal degradation by the PHD-pVHL pathway to reduce DNA hydroxymethylation
Q37699330TRIM11, a direct target of miR-24-3p, promotes cell proliferation and inhibits apoptosis in colon cancer
Q100455154Targeting the HIF2-VEGF axis in renal cell carcinoma
Q38668940Targeting the RhoGTPase/ROCK pathway for the treatment of VHL/HIF pathway-driven cancers.
Q54112866Targeting the mevalonate pathway suppresses VHL-deficient CC-RCC through a HIF-dependent mechanism.
Q38601568Targeting tumor suppressor genes for cancer therapy
Q93102798The Complex Interplay between Metabolic Reprogramming and Epigenetic Alterations in Renal Cell Carcinoma
Q92254395The E3 ligase VHL promotes follicular helper T cell differentiation via glycolytic-epigenetic control
Q38672574The Elephant in the Room: The Role of Microtubules in Cancer
Q50026055The HIF and other quandaries in VHL disease
Q51091490The epigenetic landscape of clear-cell renal cell carcinoma
Q39021700The epigenetic landscape of renal cancer
Q28079015The ever-expanding role of HIF in tumour and stromal biology
Q57903458The glutathione redox system is essential to prevent ferroptosis caused by impaired lipid metabolism in clear cell renal cell carcinoma
Q37109549The multifaceted role of autophagy in tumor evasion from immune surveillance
Q51767200The two glycolytic markers GLUT1 and MCT1 correlate with tumor grade and survival in clear-cell renal cell carcinoma
Q91809745The β2-adrenergic receptor antagonist ICI-118,551 blocks the constitutively activated HIF signalling in hemangioblastomas from von Hippel-Lindau disease
Q38871343Therapeutic Strategies for Hereditary Kidney Cancer
Q26796702Therapeutic challenges in renal cell carcinoma
Q90353766Therapies targeting the signal pathways of pheochromocytoma and paraganglioma
Q39257898Transcriptome- and proteome-oriented identification of dysregulated eIF4G, STAT3, and Hippo pathways altered by PIK3CA H1047R in HER2/ER-positive breast cancer
Q37645058USP9X destabilizes pVHL and promotes cell proliferation
Q46609299Ubiquitin ligases in oncogenic transformation and cancer therapy
Q103825405Ultrasound Microbubble-Mediated VHL Regulates the Biological Behavior of Ovarian Cancer Cells
Q92060302Upregulation of miR-211 Promotes Chondrosarcoma Development via Targeting Tumor Suppressor VHL
Q54943750VHL and Hypoxia Signaling: Beyond HIF in Cancer.
Q33826897VHL deficiency augments anthracycline sensitivity of clear cell renal cell carcinomas by down-regulating ALDH2.
Q38714947VHL promotes immune response against renal cell carcinoma via NF-κB-dependent regulation of VCAM-1.
Q37166626VHLdb: A database of von Hippel-Lindau protein interactors and mutations
Q50278297Vascular Endothelial (VE)-Cadherin, Endothelial Adherens Junctions, and Vascular Disease
Q89868243Von Hippel-Lindau Disease and the Eye
Q55110633Von Hippel-Lindau Disease.
Q51738941Von Hippel-Lindau mutations disrupt vascular patterning and maturation via Notch.
Q42378385Von Hippel-Lindau regulates interleukin-32β stability in ovarian cancer cells.
Q36286769WSB1 promotes tumor metastasis by inducing pVHL degradation
Q91980552Whole-exome sequencing and immunohistochemistry findings in von Hippel-Lindau disease
Q26744312Wnt Signaling in Renal Cell Carcinoma
Q94550232ZHX2 drives cell growth and migration via activating MEK/ERK signal and induces Sunitinib resistance by regulating the autophagy in clear cell Renal Cell Carcinoma
Q37666827pVHL suppresses kinase activity of Akt in a proline-hydroxylation-dependent manner
Q89967141von Hippel-Lindau Syndrome: Genetic Study of Case With a Rare Pathogenic Variant With Optic Nerve Hemangioblastoma, a Rare Phenotypic Expression

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