Published in Blood on January 15, 2004
Natural selection on EPAS1 (HIF2alpha) associated with low hemoglobin concentration in Tibetan highlanders. Proc Natl Acad Sci U S A (2010) 3.78
Regulation of iron homeostasis by the hypoxia-inducible transcription factors (HIFs). J Clin Invest (2007) 3.40
A family with erythrocytosis establishes a role for prolyl hydroxylase domain protein 2 in oxygen homeostasis. Proc Natl Acad Sci U S A (2006) 2.90
Somatic inactivation of the PHD2 prolyl hydroxylase causes polycythemia and congestive heart failure. Blood (2007) 2.51
von Hippel-Lindau disease: a clinical and scientific review. Eur J Hum Genet (2011) 2.45
Failure to prolyl hydroxylate hypoxia-inducible factor alpha phenocopies VHL inactivation in vivo. EMBO J (2006) 2.41
Hypoxia-inducible factors in the kidney. Am J Physiol Renal Physiol (2006) 2.25
Inhibition of oxygen sensors as a therapeutic strategy for ischaemic and inflammatory disease. Nat Rev Drug Discov (2009) 2.10
Hypoxic regulation of erythropoiesis and iron metabolism. Am J Physiol Renal Physiol (2010) 1.76
Mutation of von Hippel-Lindau tumour suppressor and human cardiopulmonary physiology. PLoS Med (2006) 1.66
Novel exon 12 mutations in the HIF2A gene associated with erythrocytosis. Blood (2008) 1.59
von Hippel-Lindau mutation in mice recapitulates Chuvash polycythemia via hypoxia-inducible factor-2alpha signaling and splenic erythropoiesis. J Clin Invest (2007) 1.56
pVHL acts as an adaptor to promote the inhibitory phosphorylation of the NF-kappaB agonist Card9 by CK2. Mol Cell (2007) 1.47
Regulation of erythropoiesis by hypoxia-inducible factors. Blood Rev (2013) 1.47
The von Hippel-Lindau Chuvash mutation promotes pulmonary hypertension and fibrosis in mice. J Clin Invest (2010) 1.46
The human side of hypoxia-inducible factor. Br J Haematol (2008) 1.41
The VHL tumor suppressor in development and disease: functional studies in mice by conditional gene targeting. Semin Cell Dev Biol (2005) 1.34
Testosterone induces erythrocytosis via increased erythropoietin and suppressed hepcidin: evidence for a new erythropoietin/hemoglobin set point. J Gerontol A Biol Sci Med Sci (2013) 1.17
Clusterin is a secreted marker for a hypoxia-inducible factor-independent function of the von Hippel-Lindau tumor suppressor protein. Am J Pathol (2006) 1.16
Cardiopulmonary function in two human disorders of the hypoxia-inducible factor (HIF) pathway: von Hippel-Lindau disease and HIF-2alpha gain-of-function mutation. FASEB J (2011) 1.14
Erythrocytosis and pulmonary hypertension in a mouse model of human HIF2A gain of function mutation. J Biol Chem (2013) 1.07
Hypoxia regulates BMP4 expression in the murine spleen during the recovery from acute anemia. PLoS One (2010) 1.04
Oxygen sensing: recent insights from idiopathic erythrocytosis. Cell Cycle (2006) 1.02
VHL inactivation in renal cell carcinoma: implications for diagnosis, prognosis and treatment. Expert Rev Anticancer Ther (2008) 1.00
The effect of erythropoietin on normal and neoplastic cells. Biologics (2012) 0.98
Genetic causes of erythrocytosis and the oxygen-sensing pathway. Blood Rev (2008) 0.97
The phenotype of polycythemia due to Croatian homozygous VHL (571C>G:H191D) mutation is different from that of Chuvash polycythemia (VHL 598C>T:R200W). Haematologica (2013) 0.97
HIF-1α is a protective factor in conditional PHD2-deficient mice suffering from severe HIF-2α-induced excessive erythropoiesis. Blood (2012) 0.95
Chuvash polycythemia VHLR200W mutation is associated with down-regulation of hepcidin expression. Blood (2011) 0.94
Pulmonary artery pressure and iron deficiency in patients with upregulation of hypoxia sensing due to homozygous VHL(R200W) mutation (Chuvash polycythemia). Haematologica (2011) 0.94
Systemic VHL gene functions and the VHL disease. FEBS Lett (2012) 0.94
Complex cellular functions of the von Hippel-Lindau tumor suppressor gene: insights from model organisms. Oncogene (2011) 0.92
HIF prolyl hydroxylase inhibitors for the treatment of renal anaemia and beyond. Nat Rev Nephrol (2015) 0.92
Altered cytokine profiles in patients with Chuvash polycythemia. Am J Hematol (2009) 0.90
Decreased serum glucose and glycosylated hemoglobin levels in patients with Chuvash polycythemia: a role for HIF in glucose metabolism. J Mol Med (Berl) (2012) 0.90
The Endothelial Prolyl-4-Hydroxylase Domain 2/Hypoxia-Inducible Factor 2 Axis Regulates Pulmonary Artery Pressure in Mice. Mol Cell Biol (2016) 0.89
The heterozygote advantage of the Chuvash polycythemia VHLR200W mutation may be protection against anemia. Haematologica (2011) 0.89
Complications in children and adolescents with Chuvash polycythemia. Blood (2015) 0.88
Heights and haematology: the story of haemoglobin at altitude. Postgrad Med J (2007) 0.86
Increased size of solid organs in patients with Chuvash polycythemia and in mice with altered expression of HIF-1alpha and HIF-2alpha. J Mol Med (Berl) (2010) 0.84
Elevated homocysteine, glutathione and cysteinylglycine concentrations in patients homozygous for the Chuvash polycythemia VHL mutation. Haematologica (2008) 0.84
The von Hippel-Lindau Chuvash mutation in mice causes carotid-body hyperplasia and enhanced ventilatory sensitivity to hypoxia. J Appl Physiol (1985) (2013) 0.83
Mimicking hypoxia to treat anemia: HIF-stabilizer BAY 85-3934 (Molidustat) stimulates erythropoietin production without hypertensive effects. PLoS One (2014) 0.82
Effect of congenital upregulation of hypoxia inducible factors on percentage of fetal hemoglobin in the blood. Blood (2013) 0.82
Progress in detecting cell-surface protein receptors: the erythropoietin receptor example. Ann Hematol (2013) 0.81
Genomic analysis of high altitude adaptation: innovations and implications. Curr Sports Med Rep (2011) 0.80
Erythropoietic agents and the elderly. Semin Hematol (2008) 0.78
Genetic evidence of a precisely tuned dysregulation in the hypoxia signaling pathway during oncogenesis. Cancer Res (2014) 0.77
Iron deficiency modifies gene expression variation induced by augmented hypoxia sensing. Blood Cells Mol Dis (2013) 0.77
Association between serum concentrations of hypoxia inducible factor responsive proteins and excessive erythrocytosis in high altitude peru. High Alt Med Biol (2015) 0.77
Diagnosis and management of congenital and idiopathic erythrocytosis. Ther Adv Hematol (2012) 0.76
Hb Heathrow [β103(G5)Phe→Leu], a First Report in an Asian Patient with Erythrocytosis. Yonsei Med J (2017) 0.75
Mitochondrial DNA 10609T promotes hypoxia-induced increase of intracellular ROS and is a risk factor of high altitude polycythemia. PLoS One (2014) 0.75
The high oxygen affinity haemoglobin Nantes: a family case description. Blood Transfus (2014) 0.75
A novel biological function of soluble biglycan: Induction of erythropoietin production and polycythemia. Glycoconj J (2016) 0.75
Could hypoxia increase the prevalence of thrombotic complications in polycythemia vera? Blood Coagul Fibrinolysis (2013) 0.75
William Kaelin, Peter Ratcliffe, and Gregg Semenza receive the 2016 Albert Lasker Basic Medical Research Award. J Clin Invest (2016) 0.75
Induction of erythropoiesis by hypoxia-inducible factor prolyl hydroxylase inhibitors without promotion of tumor initiation, progression, or metastasis in a VEGF-sensitive model of spontaneous breast cancer. Hypoxia (Auckl) (2017) 0.75
The von Hippel-Lindau Chuvash mutation in mice alters cardiac substrate and high-energy phosphate metabolism. Am J Physiol Heart Circ Physiol (2016) 0.75
Molecular basis of polycythemic disorders due to aberrant hypoxia sensing and its relevance to acute leukemia. Best Pract Res Clin Haematol (2012) 0.75
Investigation of genetic disturbances in oxygen sensing and erythropoietin signaling pathways in cases of idiopathic erythrocytosis. Genet Res Int (2013) 0.75
Therapeutic targeting of the HIF oxygen-sensing pathway: Lessons learned from clinical studies. Exp Cell Res (2017) 0.75
Phase II trial of single-agent bevacizumab followed by bevacizumab plus irinotecan at tumor progression in recurrent glioblastoma. J Clin Oncol (2008) 8.79
Magnetic resonance imaging and computed tomography in emergency assessment of patients with suspected acute stroke: a prospective comparison. Lancet (2007) 8.68
Redefining endothelial progenitor cells via clonal analysis and hematopoietic stem/progenitor cell principals. Blood (2006) 8.56
Diffusion-weighted magnetic resonance imaging as a cancer biomarker: consensus and recommendations. Neoplasia (2009) 7.44
Essential role for Nix in autophagic maturation of erythroid cells. Nature (2008) 6.49
Neonatal-onset multisystem inflammatory disease responsive to interleukin-1beta inhibition. N Engl J Med (2006) 6.17
Imaging of angiogenesis: from microscope to clinic. Nat Med (2003) 5.51
Genetic evidence for high-altitude adaptation in Tibet. Science (2010) 5.37
An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist. N Engl J Med (2009) 4.97
Comparison of MRI and CT for detection of acute intracerebral hemorrhage. JAMA (2004) 4.82
Rapid cancer detection by topically spraying a γ-glutamyltranspeptidase-activated fluorescent probe. Sci Transl Med (2011) 4.72
Magnetic resonance imaging/ultrasound-fusion biopsy significantly upgrades prostate cancer versus systematic 12-core transrectal ultrasound biopsy. Eur Urol (2013) 4.64
Clearance properties of nano-sized particles and molecules as imaging agents: considerations and caveats. Nanomedicine (Lond) (2008) 4.08
Disruption of oxygen homeostasis underlies congenital Chuvash polycythemia. Nat Genet (2002) 4.06
Genital transmission of HPV in a mouse model is potentiated by nonoxynol-9 and inhibited by carrageenan. Nat Med (2007) 3.88
New strategies for fluorescent probe design in medical diagnostic imaging. Chem Rev (2010) 3.35
Germline BHD-mutation spectrum and phenotype analysis of a large cohort of families with Birt-Hogg-Dubé syndrome. Am J Hum Genet (2005) 3.26
JAK2 mutation 1849G>T is rare in acute leukemias but can be found in CMML, Philadelphia chromosome-negative CML, and megakaryocytic leukemia. Blood (2005) 3.24
Selective molecular imaging of viable cancer cells with pH-activatable fluorescence probes. Nat Med (2008) 3.15
Prostate cancer: value of multiparametric MR imaging at 3 T for detection--histopathologic correlation. Radiology (2010) 3.12
Imaging prostate cancer: a multidisciplinary perspective. Radiology (2007) 2.79
Acquired uniparental disomy of chromosome 9p is a frequent stem cell defect in polycythemia vera. Exp Hematol (2002) 2.77
Cancer cell-selective in vivo near infrared photoimmunotherapy targeting specific membrane molecules. Nat Med (2011) 2.76
Polycythemia vera is not initiated by JAK2V617F mutation. Exp Hematol (2007) 2.75
Somatic HIF2A gain-of-function mutations in paraganglioma with polycythemia. N Engl J Med (2012) 2.74
Ascorbate in pharmacologic concentrations selectively generates ascorbate radical and hydrogen peroxide in extracellular fluid in vivo. Proc Natl Acad Sci U S A (2007) 2.71
Utility of multiparametric magnetic resonance imaging suspicion levels for detecting prostate cancer. J Urol (2013) 2.62
Overview of dynamic contrast-enhanced MRI in prostate cancer diagnosis and management. AJR Am J Roentgenol (2012) 2.59
Correlation of magnetic resonance imaging tumor volume with histopathology. J Urol (2012) 2.55
In vivo molecular imaging of cancer with a quenching near-infrared fluorescent probe using conjugates of monoclonal antibodies and indocyanine green. Cancer Res (2009) 2.51
Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. J Neurosurg Spine (2010) 2.49
Simultaneous multicolor imaging of five different lymphatic basins using quantum dots. Nano Lett (2007) 2.46
11C-Acetate PET/CT in localized prostate cancer: a study with MRI and histopathologic correlation. J Nucl Med (2012) 2.42
Hypoxia-inducible factor-1 deficiency results in dysregulated erythropoiesis signaling and iron homeostasis in mouse development. J Biol Chem (2006) 2.39
Is apparent diffusion coefficient associated with clinical risk scores for prostate cancers that are visible on 3-T MR images? Radiology (2010) 2.38
Prostate cancer: can multiparametric MR imaging help identify patients who are candidates for active surveillance? Radiology (2013) 2.35
The relationship between renal tumor size and metastases in patients with von Hippel-Lindau disease. J Urol (2004) 2.32
Hypo-functional SLC26A4 variants associated with nonsyndromic hearing loss and enlargement of the vestibular aqueduct: genotype-phenotype correlation or coincidental polymorphisms? Hum Mutat (2009) 2.25
Neurofibromatosis type 2. Lancet (2009) 2.24
Toxicity of organic fluorophores used in molecular imaging: literature review. Mol Imaging (2009) 2.21
MRI of tumor angiogenesis. J Magn Reson Imaging (2007) 2.19
Identification of a human mutation of DMT1 in a patient with microcytic anemia and iron overload. Blood (2004) 2.12
D'Amico risk stratification correlates with degree of suspicion of prostate cancer on multiparametric magnetic resonance imaging. J Urol (2011) 2.10
X-linked clonality testing: interpretation and limitations. Blood (2007) 2.04
Endothelin-1, vascular endothelial growth factor and systolic pulmonary artery pressure in patients with Chuvash polycythemia. Haematologica (2006) 2.04
In vivo diagnosis of epidermal growth factor receptor expression using molecular imaging with a cocktail of optically labeled monoclonal antibodies. Clin Cancer Res (2007) 2.01
Congenital polycythemias/erythrocytoses. Haematologica (2005) 1.99
Regulated expression of microRNAs in normal and polycythemia vera erythropoiesis. Exp Hematol (2007) 1.98
Accuracy of multiparametric magnetic resonance imaging in confirming eligibility for active surveillance for men with prostate cancer. Cancer (2013) 1.97
Hematopoiesis is not clonal in healthy elderly women. Blood (2008) 1.95
Pathophysiology of primary spinal syringomyelia. J Neurosurg Spine (2012) 1.92
Dynamic contrast-enhanced MRI of prostate cancer at 3 T: a study of pharmacokinetic parameters. AJR Am J Roentgenol (2007) 1.92
Imaging localized prostate cancer: current approaches and new developments. AJR Am J Roentgenol (2009) 1.89
Initial clinical experience with real-time transrectal ultrasonography-magnetic resonance imaging fusion-guided prostate biopsy. BJU Int (2007) 1.86
Dendrimer-based nanoprobe for dual modality magnetic resonance and fluorescence imaging. Nano Lett (2006) 1.80
Successful and safe perfusion of the primate brainstem: in vivo magnetic resonance imaging of macromolecular distribution during infusion. J Neurosurg (2002) 1.79
Spectral fluorescence molecular imaging of lung metastases targeting HER2/neu. Clin Cancer Res (2007) 1.76
Imaging techniques for prostate cancer: implications for focal therapy. Nat Rev Urol (2009) 1.76
Target-cancer-cell-specific activatable fluorescence imaging probes: rational design and in vivo applications. Acc Chem Res (2010) 1.74
Percutaneous radio frequency ablation of small renal tumors: initial results. J Urol (2002) 1.74
Multimodal nanoprobes for radionuclide and five-color near-infrared optical lymphatic imaging. ACS Nano (2007) 1.70
Clinical, genetic and radiographic analysis of 108 patients with von Hippel-Lindau disease (VHL) manifested by pancreatic neuroendocrine neoplasms (PNETs). Surgery (2007) 1.67
Radiologic manifestations of Proteus syndrome. Radiographics (2004) 1.66
In vivo molecular imaging to diagnose and subtype tumors through receptor-targeted optically labeled monoclonal antibodies. Neoplasia (2007) 1.65
Mutations in the VHL gene in sporadic apparently congenital polycythemia. Blood (2002) 1.65
A target cell-specific activatable fluorescence probe for in vivo molecular imaging of cancer based on a self-quenched avidin-rhodamine conjugate. Cancer Res (2007) 1.64
User-friendly software for the analysis of brain lesions (ABLe). Comput Methods Programs Biomed (2007) 1.63
Genetic association analysis of chronic mountain sickness in an Andean high-altitude population. Haematologica (2005) 1.59
Long-term natural history of neurofibromatosis Type 2-associated intracranial tumors. J Neurosurg (2012) 1.59
H-type dimer formation of fluorophores: a mechanism for activatable, in vivo optical molecular imaging. ACS Chem Biol (2009) 1.56
Markedly enhanced permeability and retention effects induced by photo-immunotherapy of tumors. ACS Nano (2012) 1.56
Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: implications for treatment. J Neurosurg (2006) 1.55
Lymphatic drainage imaging of breast cancer in mice by micro-magnetic resonance lymphangiography using a nano-size paramagnetic contrast agent. J Natl Cancer Inst (2004) 1.55
In vivo target-specific activatable near-infrared optical labeling of humanized monoclonal antibodies. Mol Cancer Ther (2009) 1.55
Near-infrared theranostic photoimmunotherapy (PIT): repeated exposure of light enhances the effect of immunoconjugate. Bioconjug Chem (2012) 1.55
Quantification of fibrosis and osteosclerosis in myeloproliferative neoplasms: a computer-assisted image study. Leuk Res (2010) 1.54
The association of serum ferritin and transferrin receptor concentrations with mortality in women with human immunodeficiency virus infection. Haematologica (2006) 1.53
Clonal hematopoiesis in familial polycythemia vera suggests the involvement of multiple mutational events in the early pathogenesis of the disease. Blood (2003) 1.52
Copper deficiency masquerading as myelodysplastic syndrome. Blood (2002) 1.51
Quantification of brain lesions using interactive automated software. Behav Res Methods Instrum Comput (2002) 1.51
West Nile virus: pathogenesis and therapeutic options. Ann Intern Med (2004) 1.51
Real-time image-guided direct convective perfusion of intrinsic brainstem lesions. Technical note. J Neurosurg (2007) 1.51
The vestibular aqueduct: site of origin of endolymphatic sac tumors. J Neurosurg (2008) 1.49
Phase II clinical trial of cediranib in patients with metastatic castration-resistant prostate cancer. BJU Int (2013) 1.47
Normal vision despite narrowing of the optic canal in fibrous dysplasia. N Engl J Med (2002) 1.47
Macromolecular MRI contrast agents for imaging tumor angiogenesis. Eur J Radiol (2006) 1.46
Detection of JAK2 mutations in paraffin marrow biopsies by high resolution melting analysis: identification of L611S alone and in cis with V617F in polycythemia vera. Leuk Lymphoma (2012) 1.46
An enzymatically activated fluorescence probe for targeted tumor imaging. J Am Chem Soc (2007) 1.46