Published in N Engl J Med on December 18, 2008
Hypoxia and inflammation. N Engl J Med (2011) 7.71
Somatic HIF2A gain-of-function mutations in paraganglioma with polycythemia. N Engl J Med (2012) 2.74
Tumor vasculature is regulated by PHD2-mediated angiogenesis and bone marrow-derived cell recruitment. Cancer Cell (2009) 1.82
Vitamin C is dispensable for oxygen sensing in vivo. Blood (2011) 1.68
A feedback loop involving the Phd3 prolyl hydroxylase tunes the mammalian hypoxic response in vivo. Mol Cell Biol (2009) 1.63
Regulation of erythropoiesis by hypoxia-inducible factors. Blood Rev (2013) 1.47
Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer (2014) 1.33
New syndrome of paraganglioma and somatostatinoma associated with polycythemia. J Clin Oncol (2013) 1.32
Erythropoietin. Cold Spring Harb Perspect Med (2013) 1.28
A novel EPAS1/HIF2A germline mutation in a congenital polycythemia with paraganglioma. J Mol Med (Berl) (2012) 1.13
Metastatic paraganglioma. Semin Oncol (2010) 1.12
Human high-altitude adaptation: forward genetics meets the HIF pathway. Genes Dev (2014) 1.09
Novel HIF2A mutations disrupt oxygen sensing, leading to polycythemia, paragangliomas, and somatostatinomas. Blood (2013) 1.07
The role of HIF prolyl hydroxylases in tumour growth. J Cell Mol Med (2010) 1.06
PHD2 in tumour angiogenesis. Br J Cancer (2010) 1.02
CYSL-1 interacts with the O2-sensing hydroxylase EGL-9 to promote H2S-modulated hypoxia-induced behavioral plasticity in C. elegans. Neuron (2012) 1.01
SDH-related pheochromocytoma and paraganglioma. Best Pract Res Clin Endocrinol Metab (2010) 1.00
Hypoxia-inducible factor signaling in pheochromocytoma: turning the rudder in the right direction. J Natl Cancer Inst (2013) 0.98
Molecular and therapeutic advances in the diagnosis and management of malignant pheochromocytomas and paragangliomas. Oncologist (2013) 0.97
Mutation analysis of HIF prolyl hydroxylases (PHD/EGLN) in individuals with features of phaeochromocytoma and renal cell carcinoma susceptibility. Endocr Relat Cancer (2010) 0.95
HIF-1α is a protective factor in conditional PHD2-deficient mice suffering from severe HIF-2α-induced excessive erythropoiesis. Blood (2012) 0.95
Diabetic nephropathy: a disorder of oxygen metabolism? Nat Rev Nephrol (2009) 0.94
Pheochromocytoma: implications in tumorigenesis and the actual management. Minerva Endocrinol (2012) 0.93
Prolyl hydroxylase domain protein 2 (PHD2) binds a Pro-Xaa-Leu-Glu motif, linking it to the heat shock protein 90 pathway. J Biol Chem (2013) 0.93
EGLN1 Inhibition and Rerouting of α-Ketoglutarate Suffice for Remote Ischemic Protection. Cell (2016) 0.93
Distinct deregulation of the hypoxia inducible factor by PHD2 mutants identified in germline DNA of patients with polycythemia. Haematologica (2011) 0.92
Pheochromocytoma and paraganglioma. Prog Brain Res (2010) 0.91
Combined blockade of signalling pathways shows marked anti-tumour potential in phaeochromocytoma cell lines. J Mol Endocrinol (2012) 0.90
A knock-in mouse model of human PHD2 gene-associated erythrocytosis establishes a haploinsufficiency mechanism. J Biol Chem (2013) 0.89
Rare germline mutations identified by targeted next-generation sequencing of susceptibility genes in pheochromocytoma and paraganglioma. J Clin Endocrinol Metab (2014) 0.89
Paragangliomas/Pheochromocytomas: clinically oriented genetic testing. Int J Endocrinol (2014) 0.89
Current views on cell metabolism in SDHx-related pheochromocytoma and paraganglioma. Endocr Relat Cancer (2014) 0.88
Integrity of the prolyl hydroxylase domain protein 2:erythropoietin pathway in aging mice. Blood Cells Mol Dis (2010) 0.88
Clinical iron deficiency disturbs normal human responses to hypoxia. J Clin Invest (2016) 0.87
An update on the genetics of pheochromocytoma. J Hum Hypertens (2012) 0.87
Adrenal medullary hyperplasia is a precursor lesion for pheochromocytoma in MEN2 syndrome. Neoplasia (2014) 0.87
Massive extra-adrenal retroperitoneal paraganglioma: pre-operative embolization and resection. Dig Dis Sci (2009) 0.86
Isolated erythrocytosis: study of 67 patients and identification of three novel germ-line mutations in the prolyl hydroxylase domain protein 2 (PHD2) gene. Haematologica (2011) 0.85
PHD2 mutation and congenital erythrocytosis with paraganglioma. N Engl J Med (2009) 0.84
Rethinking pheochromocytomas and paragangliomas from a genomic perspective. Oncogene (2015) 0.83
Familial pheochromocytomas and paragangliomas. Mol Cell Endocrinol (2013) 0.83
First report of bilateral pheochromocytoma in the clinical spectrum of HIF2A-related polycythemia-paraganglioma syndrome. J Clin Endocrinol Metab (2013) 0.83
The genetic basis of pheochromocytoma and paraganglioma: implications for management. Urology (2014) 0.83
Advances in understanding the pathogenesis of primary familial and congenital polycythaemia. Br J Haematol (2010) 0.83
Succinate dehydrogenase gene mutations are strongly associated with paraganglioma of the organ of Zuckerkandl. Endocr Relat Cancer (2010) 0.82
Immunohistochemical markers of the hypoxic response can identify malignancy in phaeochromocytomas and paragangliomas and optimize the detection of tumours with VHL germline mutations. Br J Cancer (2012) 0.82
Cell cycle progression in response to oxygen levels. Cell Mol Life Sci (2014) 0.82
Neuronal apoptosis by prolyl hydroxylation: implication in nervous system tumours and the Warburg conundrum. J Cell Mol Med (2009) 0.82
Prevalence of germline mutations in patients with pheochromocytoma or abdominal paraganglioma and sporadic presentation: a population-based study in Western Sweden. World J Surg (2012) 0.82
Integrative genetic characterization and phenotype correlations in pheochromocytoma and paraganglioma tumours. PLoS One (2014) 0.81
The role of PHD2 mutations in the pathogenesis of erythrocytosis. Hypoxia (Auckl) (2014) 0.80
Analysis of HIF-1a and its regulator, PHD2, in retroperitoneal sarcomas: clinico-pathologic implications. Cancer Biol Ther (2009) 0.80
Whole-exome sequencing defines the mutational landscape of pheochromocytoma and identifies KMT2D as a recurrently mutated gene. Genes Chromosomes Cancer (2015) 0.80
Novel compound VHL heterozygosity (VHL T124A/L188V) associated with congenital polycythaemia. Br J Haematol (2013) 0.79
PHD2: from hypoxia regulation to disease progression. Hypoxia (Auckl) (2016) 0.78
Genetic evidence of a precisely tuned dysregulation in the hypoxia signaling pathway during oncogenesis. Cancer Res (2014) 0.77
Therapeutic inhibition of prolyl hydroxylase domain-containing enzymes in surgery: putative applications and challenges. Hypoxia (Auckl) (2015) 0.76
Erythrocytosis due to PHD2 Mutations: A Review of Clinical Presentation, Diagnosis, and Genetics. Case Rep Hematol (2016) 0.75
Bioinformatic Challenges in Clinical Diagnostic Application of Targeted Next Generation Sequencing: Experience from Pheochromocytoma. PLoS One (2015) 0.75
Computational analysis of prolyl hydroxylase domain-containing protein 2 (PHD2) mutations promoting polycythemia insurgence in humans. Sci Rep (2016) 0.75
Identification of non-coding genetic variants in samples from hypoxemic respiratory disease patients that affect the transcriptional response to hypoxia. Nucleic Acids Res (2016) 0.75
Functional Imaging Signature of Patients Presenting with Polycythemia/Paraganglioma Syndromes. J Nucl Med (2017) 0.75
Role of SDHAF2 and SDHD in von Hippel-Lindau associated pheochromocytomas. World J Surg (2014) 0.75
Arginine methylation an emerging regulator of protein function. Mol Cell (2005) 6.49
HIF prolyl-hydroxylase 2 is the key oxygen sensor setting low steady-state levels of HIF-1alpha in normoxia. EMBO J (2003) 6.44
Hypoxia-induced autophagy is mediated through hypoxia-inducible factor induction of BNIP3 and BNIP3L via their BH3 domains. Mol Cell Biol (2009) 4.95
Target RNA motif and target mRNAs of the Quaking STAR protein. Nat Struct Mol Biol (2005) 3.70
Birt-Hogg-Dubé syndrome: diagnosis and management. Lancet Oncol (2009) 3.60
A SUMOylation-defective MITF germline mutation predisposes to melanoma and renal carcinoma. Nature (2011) 3.53
JunD reduces tumor angiogenesis by protecting cells from oxidative stress. Cell (2004) 3.30
Genetic testing in pheochromocytoma or functional paraganglioma. J Clin Oncol (2005) 3.20
RNA-binding proteins in human genetic disease. Trends Genet (2008) 3.14
ERK implication in cell cycle regulation. Biochim Biophys Acta (2006) 2.96
The role of erk1 and erk2 in multiple stages of T cell development. Immunity (2005) 2.96
Knockout of ERK1 MAP kinase enhances synaptic plasticity in the striatum and facilitates striatal-mediated learning and memory. Neuron (2002) 2.93
Hypoxia and cancer. J Mol Med (Berl) (2007) 2.89
An antioxidant response phenotype shared between hereditary and sporadic type 2 papillary renal cell carcinoma. Cancer Cell (2011) 2.78
Hypoxia-inducible carbonic anhydrase IX and XII promote tumor cell growth by counteracting acidosis through the regulation of the intracellular pH. Cancer Res (2009) 2.62
A proteomic analysis of arginine-methylated protein complexes. Mol Cell Proteomics (2003) 2.59
Crypt-restricted proliferation and commitment to the Paneth cell lineage following Apc loss in the mouse intestine. Development (2005) 2.56
Transcriptional regulation of the Vascular Endothelial Growth Factor gene--a concert of activating factors. Cardiovasc Res (2005) 2.52
Hypoxia signalling controls metabolic demand. Curr Opin Cell Biol (2007) 2.49
von Hippel-Lindau disease: a clinical and scientific review. Eur J Hum Genet (2011) 2.45
Hypoxia-induced autophagy: cell death or cell survival? Curr Opin Cell Biol (2009) 2.45
Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patients. EMBO J (2010) 2.43
RNF8- and RNF168-dependent degradation of KDM4A/JMJD2A triggers 53BP1 recruitment to DNA damage sites. EMBO J (2012) 2.34
Intravenous scAAV9 delivery of a codon-optimized SMN1 sequence rescues SMA mice. Hum Mol Genet (2010) 2.26
The Y deletion gr/gr and susceptibility to testicular germ cell tumor. Am J Hum Genet (2005) 2.26
BRCA1 supports XIST RNA concentration on the inactive X chromosome. Cell (2002) 2.24
Tudor domains bind symmetrical dimethylated arginines. J Biol Chem (2005) 2.23
Genome-wide linkage screen for testicular germ cell tumour susceptibility loci. Hum Mol Genet (2006) 2.23
Erk kinases link pre-B cell receptor signaling to transcriptional events required for early B cell expansion. Immunity (2008) 2.16
Tumour hypoxia induces a metabolic shift causing acidosis: a common feature in cancer. J Cell Mol Med (2009) 2.13
Sam68, the KH domain-containing superSTAR. Biochim Biophys Acta (2003) 2.08
Rituximab efficacy and safety in adult splenectomy candidates with chronic immune thrombocytopenic purpura: results of a prospective multicenter phase 2 study. Blood (2008) 2.07
CD147 subunit of lactate/H+ symporters MCT1 and hypoxia-inducible MCT4 is critical for energetics and growth of glycolytic tumors. Proc Natl Acad Sci U S A (2011) 2.06
Methylation of Tat by PRMT6 regulates human immunodeficiency virus type 1 gene expression. J Virol (2005) 2.02
Vasculitides associated with malignancies: analysis of sixty patients. Arthritis Rheum (2007) 1.97
The extracellular signal-regulated kinase isoform ERK1 is specifically required for in vitro and in vivo adipogenesis. Diabetes (2005) 1.96
Identification of two Sp1 phosphorylation sites for p42/p44 mitogen-activated protein kinases: their implication in vascular endothelial growth factor gene transcription. J Biol Chem (2002) 1.94
Disrupting proton dynamics and energy metabolism for cancer therapy. Nat Rev Cancer (2013) 1.93
Induction of hypoxia-inducible factor-1alpha by transcriptional and translational mechanisms. J Biol Chem (2002) 1.90
Sam68 RNA binding protein is an in vivo substrate for protein arginine N-methyltransferase 1. Mol Biol Cell (2003) 1.87
HIF-1alpha and CA IX staining in invasive breast carcinomas: prognosis and treatment outcome. Int J Cancer (2007) 1.86
Mitotic catastrophe constitutes a special case of apoptosis whose suppression entails aneuploidy. Oncogene (2004) 1.79
Smurf1 regulates tumor cell plasticity and motility through degradation of RhoA leading to localized inhibition of contractility. J Cell Biol (2006) 1.76
PHDs overactivation during chronic hypoxia "desensitizes" HIFalpha and protects cells from necrosis. Proc Natl Acad Sci U S A (2008) 1.72
New implications for the QUAKING RNA binding protein in human disease. J Neurosci Res (2008) 1.71
The International Testicular Cancer Linkage Consortium: a clinicopathologic descriptive analysis of 461 familial malignant testicular germ cell tumor kindred. Urol Oncol (2009) 1.70
MEK1-ERK2 signaling pathway protects myocardium from ischemic injury in vivo. Circulation (2004) 1.69
Germline BAP1 mutations predispose to renal cell carcinomas. Am J Hum Genet (2013) 1.69
Oxygen, a source of life and stress. FEBS Lett (2007) 1.67
Rapid detection of novel BRCA1 rearrangements in high-risk breast-ovarian cancer families using multiplex PCR of short fluorescent fragments. Hum Mutat (2002) 1.66
Protection of p27(Kip1) mRNA by quaking RNA binding proteins promotes oligodendrocyte differentiation. Nat Neurosci (2004) 1.62
Single and combined silencing of ERK1 and ERK2 reveals their positive contribution to growth signaling depending on their expression levels. Mol Cell Biol (2007) 1.62
Prognostic value of early 18 fluorodeoxyglucose positron emission tomography and gallium-67 scintigraphy in aggressive lymphoma: a prospective comparative study. Leuk Lymphoma (2006) 1.62
Hypoxia and energetic tumour metabolism. Curr Opin Genet Dev (2010) 1.61
Occupations and industries in France at high risk for pleural mesothelioma: A population-based case-control study (1998-2002). Am J Ind Med (2010) 1.61
High frequency of TP53 mutation in BRCA1 and sporadic basal-like carcinomas but not in BRCA1 luminal breast tumors. Cancer Res (2009) 1.61
Arginine methylation of MRE11 by PRMT1 is required for DNA damage checkpoint control. Genes Dev (2005) 1.61
SAM68 regulates neuronal activity-dependent alternative splicing of neurexin-1. Cell (2011) 1.60
Defining the RGG/RG motif. Mol Cell (2013) 1.59
HIF at a glance. J Cell Sci (2009) 1.55
Cancer incidence within a cohort occupationally exposed to asbestos: a study of dose--response relationships. Occup Environ Med (2011) 1.51
BRCA1 is required for postreplication repair after UV-induced DNA damage. Mol Cell (2011) 1.48
Tyrosine phosphorylation of sam68 by breast tumor kinase regulates intranuclear localization and cell cycle progression. J Biol Chem (2005) 1.47
The formin homology 1 domain modulates the actin nucleation and bundling activity of Arabidopsis FORMIN1. Plant Cell (2005) 1.46
The hypoxia-inducible-factor hydroxylases bring fresh air into hypoxia signalling. EMBO Rep (2006) 1.43
Treatment of von Hippel-Lindau retinal hemangioblastoma by the vascular endothelial growth factor receptor inhibitor SU5416 is more effective for associated macular edema than for hemangioblastomas. Am J Ophthalmol (2003) 1.42
Nuclear retention of MBP mRNAs in the quaking viable mice. Neuron (2002) 1.41
A functional role for the p62-ERK1 axis in the control of energy homeostasis and adipogenesis. EMBO Rep (2010) 1.40
A mouse PRMT1 null allele defines an essential role for arginine methylation in genome maintenance and cell proliferation. Mol Cell Biol (2009) 1.40
Protein interfaces in signaling regulated by arginine methylation. Sci STKE (2005) 1.39
X-chromosome genetics and human cancer. Nat Rev Cancer (2004) 1.39
FLI1 monoallelic expression combined with its hemizygous loss underlies Paris-Trousseau/Jacobsen thrombopenia. J Clin Invest (2004) 1.39
Arrest-defective-1 protein, an acetyltransferase, does not alter stability of hypoxia-inducible factor (HIF)-1alpha and is not induced by hypoxia or HIF. J Biol Chem (2005) 1.38
Sam68 regulates translation of target mRNAs in male germ cells, necessary for mouse spermatogenesis. J Cell Biol (2009) 1.36
The nuclear tyrosine kinase BRK/Sik phosphorylates and inhibits the RNA-binding activities of the Sam68-like mammalian proteins SLM-1 and SLM-2. J Biol Chem (2004) 1.36
Inactivation of BHD in sporadic renal tumors. Cancer Res (2003) 1.36
Loss of CARM1 results in hypomethylation of thymocyte cyclic AMP-regulated phosphoprotein and deregulated early T cell development. J Biol Chem (2004) 1.35
Signalling via the hypoxia-inducible factor-1alpha requires multiple posttranslational modifications. Cell Signal (2005) 1.35
Primary intrathoracic synovial sarcoma: a clinicopathologic study of 40 t(X;18)-positive cases from the French Sarcoma Group and the Mesopath Group. Am J Surg Pathol (2005) 1.35
Extracellular signal-regulated kinases phosphorylate mitogen-activated protein kinase phosphatase 3/DUSP6 at serines 159 and 197, two sites critical for its proteasomal degradation. Mol Cell Biol (2005) 1.34
Retinal hemangioblastoma in von Hippel-Lindau disease: a clinical and molecular study. Invest Ophthalmol Vis Sci (2002) 1.33
Raf-1-induced growth arrest in human mammary epithelial cells is p16-independent and is overcome in immortal cells during conversion. Oncogene (2002) 1.32
Arginine methylation of the human immunodeficiency virus type 1 Tat protein by PRMT6 negatively affects Tat Interactions with both cyclin T1 and the Tat transactivation region. J Virol (2007) 1.31
ERK nuclear translocation is dimerization-independent but controlled by the rate of phosphorylation. J Biol Chem (2009) 1.31
Harnessing the hypoxia-inducible factor in cancer and ischemic disease. Biochem Pharmacol (2006) 1.31
The GAR motif of 53BP1 is arginine methylated by PRMT1 and is necessary for 53BP1 DNA binding activity. Cell Cycle (2005) 1.30
PML nuclear bodies are highly organised DNA-protein structures with a function in heterochromatin remodelling at the G2 phase. J Cell Sci (2006) 1.30
Inhibition of prolyl hydroxylase domain proteins promotes therapeutic revascularization. Circulation (2009) 1.29
p44 mitogen-activated protein kinase (extracellular signal-regulated kinase 1)-dependent signaling contributes to epithelial skin carcinogenesis. Cancer Res (2006) 1.27
Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations. Hum Mol Genet (2011) 1.27
ERK2 but not ERK1 plays a key role in hepatocyte replication: an RNAi-mediated ERK2 knockdown approach in wild-type and ERK1 null hepatocytes. Hepatology (2007) 1.26
Regulation of tumor cell motility by ERK mitogen-activated protein kinases. Ann N Y Acad Sci (2004) 1.25
EphrinA1 inactivates integrin-mediated vascular smooth muscle cell spreading via the Rac/PAK pathway. J Cell Sci (2003) 1.24
HIF-1: master and commander of the hypoxic world. A pharmacological approach to its regulation by siRNAs. Biochem Pharmacol (2004) 1.22
TLR2 and RIP2 pathways mediate autophagy of Listeria monocytogenes via extracellular signal-regulated kinase (ERK) activation. J Biol Chem (2011) 1.21