Standout Papers
Citation Impact
Citing Papers
Histone demethylase KDM6A directly senses oxygen to control chromatin and cell fate
2019 StandoutScienceNobel
Gene set enrichment analysis: A knowledge-based approach for interpreting genome-wide expression profiles
2005 Standout
A family with erythrocytosis establishes a role for prolyl hydroxylase domain protein 2 in oxygen homeostasis
2006
Targeting of HIF-α to the von Hippel-Lindau Ubiquitylation Complex by O 2 -Regulated Prolyl Hydroxylation
2001 StandoutScienceNobel
HIFα Targeted for VHL-Mediated Destruction by Proline Hydroxylation: Implications for O 2 Sensing
2001 StandoutScienceNobel
A noninflammatory mRNA vaccine for treatment of experimental autoimmune encephalomyelitis
2021 StandoutScienceNobel
Conserved N-terminal cysteine dioxygenases transduce responses to hypoxia in animals and plants
2019 StandoutScienceNobel
A Conserved Family of Prolyl-4-Hydroxylases That Modify HIF
2001 StandoutScience
( R )-2-Hydroxyglutarate Is Sufficient to Promote Leukemogenesis and Its Effects Are Reversible
2013 StandoutScienceNobel
The Myeloma Drug Lenalidomide Promotes the Cereblon-Dependent Destruction of Ikaros Proteins
2013 StandoutScienceNobel
Gene expression profiling in a renal cell carcinoma cell line: dissecting VHL and hypoxia-dependent pathways.
2003 StandoutNobel
Targeted Replacement of Hypoxia-Inducible Factor-1α by a Hypoxia-Inducible Factor-2α Knock-in Allele Promotes Tumor Growth
2005
Neuronal apoptosis linked to EglN3 prolyl hydroxylase and familial pheochromocytoma genes: Developmental culling and cancer
2005 StandoutNobel
Inhibitory PAS Domain Protein (IPAS) Is a Hypoxia-inducible Splicing Variant of the Hypoxia-inducible Factor-3α Locus
2002
Cancer and Altered Metabolism: Potential Importance of Hypoxia-Inducible Factor and 2-Oxoglutarate-Dependent Dioxygenases
2011 StandoutNobel
Suppression of Hypoxia-Inducible Factor 2α Restores p53 Activity via Hdm2 and Reverses Chemoresistance of Renal Carcinoma Cells
2009
Regulation of tyrosine hydroxylase promoter activity by the von Hippel–Lindau tumor suppressor protein and hypoxia‐inducible transcription factors
2003
Cell-specific Regulation of Hypoxia-inducible Factor (HIF)-1α and HIF-2α Stabilization and Transactivation in a Graded Oxygen Environment
2006
Interaction of Hydroxylated Collagen IV with the von Hippel-Lindau Tumor Suppressor
2007 StandoutNobel
von Hippel-Lindau Tumor Suppressor Protein Regulates the Assembly of Intercellular Junctions in Renal Cancer Cells through Hypoxia-Inducible Factor–Independent Mechanisms
2006
HIF prolyl and asparaginyl hydroxylases in the biological response to intracellular O2 levels
2003 StandoutNobel
HIF overexpression correlates with biallelic loss of fumarate hydratase in renal cancer: Novel role of fumarate in regulation of HIF stability
2005 StandoutNobel
On-target efficacy of a HIF-2α antagonist in preclinical kidney cancer models
2016 StandoutNatureNobel
von Hippel–Lindau mutation in mice recapitulates Chuvash polycythemia via hypoxia-inducible factor-2α signaling and splenic erythropoiesis
2007
Activation of the HIF pathway in cancer
2001
Hallmarks of Cancer: The Next Generation
2011 Standout
Hypoxia and intra-complex genetic suppressors rescue complex I mutants by a shared mechanism
2024 StandoutNobel
Differential Roles of Hypoxia-Inducible Factor 1α (HIF-1α) and HIF-2α in Hypoxic Gene Regulation
2003
Sequence Determinants in Hypoxia-inducible Factor-1α for Hydroxylation by the Prolyl Hydroxylases PHD1, PHD2, and PHD3
2002
Inhibition of Hypoxia-Inducible Factor Is Sufficient for Growth Suppression of VHL−/− Tumors
2004
Hypoxia-Inducible Factor Linked to Differential Kidney Cancer Risk Seen with Type 2A and Type 2B VHL Mutations
2007 StandoutNobel
Hypoxia — a key regulatory factor in tumour growth
2002 Standout
HIF-1α binding to VHL is regulated by stimulus-sensitive proline hydroxylation
2001
Regulation and Destabilization of HIF-1α by ARD1-Mediated Acetylation
2002
A role for mitochondrial enzymes in inherited neoplasia and beyond
2003
Rac1 Activity Is Required for the Activation of Hypoxia-inducible Factor 1
2001 StandoutNobel
Suppression of tumor growth through disruption of hypoxia-inducible transcription
2000 StandoutNobel
Germline mutations in the von Hippel-Lindau (VHL) gene in patients from Poland: disease presentation in patients with deletions of the entire VHL gene
2002
Hsp90 Regulates a von Hippel Lindau-independent Hypoxia-inducible Factor-1α-degradative Pathway
2002
C. elegans EGL-9 and Mammalian Homologs Define a Family of Dioxygenases that Regulate HIF by Prolyl Hydroxylation
2001 StandoutNobel
MUF1, A Novel Elongin BC-interacting Leucine-rich Repeat Protein That Can Assemble with Cul5 and Rbx1 to Reconstitute a Ubiquitin Ligase
2001
pVHL Acts as an Adaptor to Promote the Inhibitory Phosphorylation of the NF-κB Agonist Card9 by CK2
2007 StandoutNobel
Common conformational changes induced in type 2 picornavirus IRESs by cognate trans-acting factors
2011
Induction of hypervascularity without leakage or inflammation in transgenic mice overexpressing hypoxia-inducible factor-1α
2001
The contribution of VHL substrate binding and HIF1-α to the phenotype of VHL loss in renal cell carcinoma
2002
Structural basis for the recognition of hydroxyproline in HIF-1α by pVHL
2002 StandoutNatureNobel
Degradation of p53 by adenovirus E4orf6 and E1B55K proteins occurs via a novel mechanism involving a Cullin-containing complex
2001 StandoutNobel
The von Hippel–Lindau tumour suppressor protein: O2 sensing and cancer
2008 StandoutNobel
FIH-1 is an asparaginyl hydroxylase enzyme that regulates the transcriptional activity of hypoxia-inducible factor
2002 Standout
Targeting HIF-1 for cancer therapy
2003 StandoutNobel
The von Hippel–Lindau Tumor Suppressor Gene
2001 StandoutNobel
The pressure rises: update on the genetics of phaeochromocytoma
2002
Analysis of von Hippel–Lindau Hereditary Cancer Syndrome: Implications of Oxygen Sensing
2004 StandoutNobel
Recent Insights into the Molecular Pathogenesis of Pheochromocytoma and Paraganglioma
2006 StandoutNobel
Von Hippel‐Lindau gene alterations in sporadic benign and malignant pheochromocytomas
2003
Molecular basis of the VHL hereditary cancer syndrome
2002 StandoutNobel
HIF‐1‐dependent transcriptional activity is required for oxygen‐mediated HIF‐1α degradation
2001
Molecular Mechanism of Hypoxia-inducible Factor 1α-p300 Interaction
2001
Transformation by the (R)-enantiomer of 2-hydroxyglutarate linked to EGLN activation
2012 StandoutNatureNobel
p53 Stabilization and Transactivation by a von Hippel-Lindau Protein
2006
Von Hippel‐Lindau disease
2003
Role of Exon 2-encoded β-Domain of the von Hippel-Lindau Tumor Suppressor Protein
2001
The von Hippel–Lindau tumor suppressor protein
2001 StandoutNobel
The lore of the RINGs: substrate recognition and catalysis by ubiquitin ligases
2000
Ubiquitination of a Novel Deubiquitinating Enzyme Requires Direct Binding to von Hippel-Lindau Tumor Suppressor Protein
2002
Hypoxia-inducible Factor and Its Biomedical Relevance
2003
Oxygen sensing by HIF hydroxylases
2004 StandoutNobel
The VHL tumor suppressor inhibits expression of the IGF1R and its loss induces IGF1R upregulation in human clear cell renal carcinoma
2007
Collagen matrix assembly is driven by the interaction of von Hippel–Lindau tumor suppressor protein with hydroxylated collagen IV alpha 2
2007
HIF-1 and mechanisms of hypoxia sensing
2001 StandoutNobel
The von Hippel-Lindau tumor suppressor gene protects cells from UV-mediated apoptosis
2000
von Hippel–Lindau tumor suppressor: not only HIF's executioner
2004
Contrasting effects on HIF-1alpha regulation by disease-causing pVHL mutations correlate with patterns of tumourigenesis in von Hippel-Lindau disease
2001
Disruption of oxygen homeostasis underlies congenital Chuvash polycythemia
2002 StandoutNobel
Understanding pathogenesis and therapy of multiple sclerosis via animal models: 70 years of merits and culprits in experimental autoimmune encephalomyelitis research
2006
Pathogenetic mechanisms of polycythemia vera and congenital polycythemic disorders
2001
von Hippel-Lindau disease
2003 Standout
HIF hydroxylation and the mammalian oxygen-sensing pathway
2003 StandoutNobel
The von Hippel-Lindau Tumor Suppressor Protein Mediates Ubiquitination of Activated Atypical Protein Kinase C
2001
Inhibition of HIF2α Is Sufficient to Suppress pVHL-Defective Tumor Growth
2003 StandoutNobel
Structure of mammalian eIF3 in the context of the 43S preinitiation complex
2015 StandoutNatureNobel
A role for hypoxia and hypoxia-inducible transcription factors in tumor physiology
2002
Failure to prolyl hydroxylate hypoxia‐inducible factor α phenocopies VHL inactivation in vivo
2006 StandoutNobel
Mobility of the von Hippel–Lindau tumour suppressor protein is regulated by kinesin-2
2008
Hypoxic gene expression and metastasis
2004
Regulation of microtubule stability by the von Hippel-Lindau tumour suppressor protein pVHL
2002
Adolescent idiopathic scoliosis
2008 Standout
VHL, the story of a tumour suppressor gene
2014
Structure of Human FIH-1 Reveals a Unique Active Site Pocket and Interaction Sites for HIF-1 and von Hippel-Lindau
2003
HIF prolyl-hydroxylase 2 is the key oxygen sensor setting low steady-state levels of HIF-1 in normoxia
2003 Standout
HIF-2α downregulation in the absence of functional VHL is not sufficient for renal cell differentiation
2007
Genetic Analysis of Pathways Regulated by the von Hippel-Lindau Tumor Suppressor in Caenorhabditis elegans
2004 StandoutNobel
Role of elongin-binding domain of von hippel lindau gene product on HuR-mediated VPF/VEGF mRNA stability in renal cell carcinoma
2005
Tumor Suppression by the von Hippel-Lindau Protein Requires Phosphorylation of the Acidic Domain
2005
Selection of Mutant CHO Cells with Constitutive Activation of the HIF System and Inactivation of the von Hippel-Lindau Tumor Suppressor
2001 StandoutNobel
The von Hippel–Lindau protein, HIF hydroxylation, and oxygen sensing
2005 StandoutNobel
Formation of Primary Cilia in the Renal Epithelium Is Regulated by the von Hippel-Lindau Tumor Suppressor Protein
2006
Leu-574 of HIF-1α Is Essential for the von Hippel-Lindau (VHL)-mediated Degradation Pathway
2002
Inhibition of HIF is necessary for tumor suppression by the von Hippel-Lindau protein
2002 StandoutNobel
Role of Prolyl Hydroxylation in Oncogenically Stabilized Hypoxia-inducible Factor-1α
2002
Activation of HIF-1α mRNA by hypoxia and iron chelator in isolated rat carotid body
2004
Identification of a deubiquitinating enzyme subfamily as substrates of the von Hippel–Lindau tumor suppressor
2002
Vascular endothelial growth factor overexpression is correlated with von Hippel‐Lindau tumor suppressor gene inactivation in patients with sporadic renal cell carcinoma
2002
The VHL protein recruits a novel KRAB-A domain protein to repress HIF-1alpha transcriptional activity
2003
Loss of pVHL is sufficient to cause HIF dysregulation in primary cells but does not promote tumor growth
2003
Independent function of two destruction domains in hypoxia-inducible factor-α chains activated by prolyl hydroxylation
2001 StandoutNobel
FIH-1: a novel protein that interacts with HIF-1α and VHL to mediate repression of HIF-1 transcriptional activity
2001 StandoutNobel
The von Hippel-Lindau Tumor Suppressor Stabilizes Novel Plant Homeodomain Protein Jade-1
2002
Hypoxia Inducible Factor Activates the Transforming Growth Factor-α/Epidermal Growth Factor Receptor Growth Stimulatory Pathway in VHL-/- Renal Cell Carcinoma Cells
2003
Multiple Splice Variants of the Human HIF-3α Locus Are Targets of the von Hippel-Lindau E3 Ubiquitin Ligase Complex
2003
An intact NEDD8 pathway is required for Cullin‐dependent ubiquitylation in mammalian cells
2002 StandoutNobel
pVHL Function Is Essential for Endothelial Extracellular Matrix Deposition
2006
Life with Oxygen
2007 StandoutScienceNobel
β-Arrestin–Mediated Localization of Smoothened to the Primary Cilium
2008 StandoutScienceNobel
Activation of HIF1α ubiquitination by a reconstituted von Hippel-Lindau (VHL) tumor suppressor complex
2000
Role of transforming growth factor-α in von Hippel– Lindau (VHL) −/− clear cell renal carcinoma cell proliferation: A possible mechanism coupling VHL tumor suppressor inactivation and tumorigenesis
2001
von Hippel-Lindau Disease
2006 StandoutNobel
HIF hydroxylation and the mammalian oxygen-sensing pathway
2003 StandoutNobel
Biochemical purification and pharmacological inhibition of a mammalian prolyl hydroxylase acting on hypoxia-inducible factor
2002 StandoutNobel
Constitutive expression of hypoxia-inducible factor-1alpha renders pancreatic cancer cells resistant to apoptosis induced by hypoxia and nutrient deprivation.
2001
PROLINE HYDROXYLATION AND GENE EXPRESSION
2005 StandoutNobel
Oxygen Sensing and HIF-1 Activation Does Not Require an Active Mitochondrial Respiratory Chain Electron-transfer Pathway
2001
Carboxyl-Terminal Transactivation Activity of Hypoxia-Inducible Factor 1α Is Governed by a von Hippel-Lindau Protein-Independent, Hydroxylation-Regulated Association with p300/CBP
2002
Hepatic vascular tumors, angiectasis in multiple organs, and impaired spermatogenesis in mice with conditional inactivation of the VHL gene.
2003
EglN3 hydroxylase stabilizes BIM-EL linking VHL type 2C mutations to pheochromocytoma pathogenesis and chemotherapy resistance
2019 StandoutNobel
Cellular adaptation to hypoxia: O 2 ‐sensing protein hydroxylases, hypoxia‐inducible transcription factors, and O 2 ‐regulated gene expression
2002
Regulation of E-cadherin Expression by VHL and Hypoxia-Inducible Factor
2006
The von Hippel-Lindau Tumor Suppressor Gene
2020
Building and remodelling Cullin–RING E3 ubiquitin ligases
2013
VHL-mediated hypoxia regulation of cyclin D1 in renal carcinoma cells.
2002
Reactivation of Hepatic EPO Synthesis in Mice After PHD Loss
2010 StandoutScienceNobel
pVHL suppresses kinase activity of Akt in a proline-hydroxylation–dependent manner
2016 StandoutScienceNobel
Nitric Oxide Impairs Normoxic Degradation of HIF-1α by Inhibition of Prolyl Hydroxylases
2003
Prolyl Hydroxylase PHD3 Activates Oxygen-dependent Protein Aggregation
2008
Involvement of Hypoxia-Inducible Factor 1 in Human Cancer.
2002 StandoutNobel
Induction of HIF-1alpha in response to hypoxia is instantaneous.
2001
Structure of an HIF-1α-pVHL Complex: Hydroxyproline Recognition in Signaling
2002 StandoutScienceNobel
pVHL Modification by NEDD8 Is Required for Fibronectin Matrix Assembly and Suppression of Tumor Development
2004 StandoutNobel
NEDD8 acts as a ‘molecular switch’ defining the functional selectivity of VHL
2008
Vascular tumors in livers with targeted inactivation of the von Hippel–Lindau tumor suppressor
2001
Regulation of colon carcinoma cell invasion by hypoxia-inducible factor 1.
2003 StandoutNobel
Intracellular localisation of human HIF-1α hydroxylases:implications for oxygen sensing
2003
Characterization of a von Hippel Lindau Pathway Involved in Extracellular Matrix Remodeling, Cell Invasion, and Angiogenesis
2006
BRCA1-IRIS promotes human tumor progression through PTEN blockade and HIF-1α activation
2018 StandoutNobel
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.
2002
GSK-3β regulates cell growth, migration, and angiogenesis via Fbw7 and USP28-dependent degradation of HIF-1α
2011
Role of the von Hippel-Lindau tumor suppressor gene in the formation of beta1-integrin fibrillar adhesions.
2002
Endogenous von Hippel-Lindau tumor suppressor protein regulates catecholaminergic phenotype in PC12 cells.
2002
von Hippel–Lindau protein binds hyperphosphorylated large subunit of RNA polymerase II through a proline hydroxylation motif and targets it for ubiquitination
2003
Angiogenesis in Endocrine Tumors
2003
Constitutive activation of hypoxia-inducible genes related to overexpression of hypoxia-inducible factor-1alpha in clear cell renal carcinomas.
2001
VHL Induces Renal Cell Differentiation and Growth Arrest through Integration of Cell-Cell and Cell-Extracellular Matrix Signaling
2001
Inactivation of the Arylhydrocarbon Receptor Nuclear Translocator (Arnt) Suppresses von Hippel-Lindau Disease-Associated Vascular Tumors in Mice
2005
Role ofVHLGene Mutation in Human Cancer
2004 StandoutNobel
Dynamic, site-specific interaction of hypoxia-inducible factor-1alpha with the von Hippel-Lindau tumor suppressor protein.
2001
Reconfigurable asymmetric protein assemblies through implicit negative design
2022 StandoutScienceNobel
The Caenorhabditis elegans hif-1 gene encodes a bHLH-PAS protein that is required for adaptation to hypoxia
2001
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
2002 StandoutNobel
Inactivation of von Hippel-Lindau Gene Induces Constitutive Phosphorylation of MET Protein in Clear Cell Renal Carcinoma
2006
BIALLELIC INACTIVATION OF THE VON HIPPEL-LINDAU TUMOR SUPPRESSOR GENE IN SPORADIC RENAL CELL CARCINOMA
2002
Hypoxic but not anoxic stabilization of HIF-1α requires mitochondrial reactive oxygen species
2002
Works of Michael A. Hoffman being referenced
von Hippel-Lindau protein mutants linked to type 2C VHL disease preserve the ability to downregulate HIF
2001
The central nervous sytem-specific myelin oligodendrocytic basic protein (MOBP) is encephalitogenic and a potential target antigen in multiple sclerosis (MS)
2000
Mechanical Modulation of Vertebral Growth in the Fusionless Treatment of Progressive Scoliosis in an Experimental Model
2006
Ubiquitination of hypoxia-inducible factor requires direct binding to the β-domain of the von Hippel–Lindau protein
2000 StandoutNobel
Sequence and structural elements that contribute to efficient encephalomyocarditis virus RNA translation
1992