W. Kaelin and J. , The von Hippel???Lindau tumour suppressor protein: O2 sensing and cancer, Nature Reviews Cancer, vol.60, issue.11, pp.865-73, 2008.
DOI : 10.1016/S1535-6108(02)00042-9

J. Pouyssegur, F. Dayan, and N. Mazure, Hypoxia signalling in cancer and approaches to enforce tumour regression, Nature, vol.9, issue.7092, pp.437-480, 2006.
DOI : 10.1038/nature04871
URL : https://hal.archives-ouvertes.fr/hal-00321054

R. Wenger, D. Stiehl, and G. Camenisch, Integration of Oxygen Signaling at the Consensus HRE, Science Signaling, vol.2005, issue.306, p.12, 2005.
DOI : 10.1126/stke.3062005re12

E. Rankin, M. Biju, Q. Liu, T. Unger, J. Rha et al., Hypoxia-inducible factor???2 (HIF-2) regulates hepatic erythropoietin in vivo, Journal of Clinical Investigation, vol.117, issue.4, pp.1068-77, 2007.
DOI : 10.1172/JCI30117

C. Rosenberger, S. Mandriota, J. Jurgensen, M. Wiesener, J. Horstrup et al., Expression of Hypoxia-Inducible Factor-1?? and -2?? in Hypoxic and Ischemic Rat Kidneys, Journal of the American Society of Nephrology, vol.13, issue.7, pp.1721-1753, 2002.
DOI : 10.1097/01.ASN.0000017223.49823.2A

C. Warnecke, Z. Zaborowska, J. Kurreck, V. Erdmann, U. Frei et al., Differentiating the functional role of hypoxia-inducible factor (HIF)-1alpha and HIF-2alpha (EPAS-1) by the use of RNA interference: erythropoietin is a HIF-2alpha target gene in Hep3B and Kelly cells, Faseb J, vol.18, issue.12, pp.1462-1466, 2004.

R. Wenger and D. Hoogewijs, Regulated oxygen sensing by protein hydroxylation in renal erythropoietin-producing cells, AJP: Renal Physiology, vol.298, issue.6, pp.1287-96, 2010.
DOI : 10.1152/ajprenal.00736.2009

S. Richard, J. Graff, J. Lindau, and F. Resche, Von Hippel-Lindau disease, The Lancet, vol.363, issue.9416, pp.1231-1235, 2004.
DOI : 10.1016/S0140-6736(04)15957-6
URL : https://hal.archives-ouvertes.fr/inserm-01402348

E. Maher, A. Webster, F. Richards, J. Green, P. Crossey et al., Phenotypic expression in von Hippel-Lindau disease: correlations with germline VHL gene mutations., Journal of Medical Genetics, vol.33, issue.4, pp.328-360, 1996.
DOI : 10.1136/jmg.33.4.328

M. Nordstrom-o-'brien, R. Van-der-luijt, E. Van-rooijen, A. Van-den-ouweland, D. Majoor-krakauer et al., Genetic analysis of von Hippel-Lindau disease, Hum Mutat, vol.31, issue.5, pp.521-558, 2010.

S. Ang, H. Chen, K. Hirota, V. Gordeuk, J. Jelinek et al., Disruption of oxygen homeostasis underlies congenital Chuvash polycythemia, Nature Genetics, vol.59, issue.4, pp.614-635, 2002.
DOI : 10.1016/0167-4889(90)90126-X

M. Al-sheikh, K. Moradkhani, M. Lopez, H. Wajcman, and C. Prehu, Disturbance in the HIF-1?? pathway associated with erythrocytosis: Further evidences brought by frameshift and nonsense mutations in the prolyl hydroxylase domain protein 2 (PHD2) gene, Blood Cells, Molecules, and Diseases, vol.40, issue.2, pp.160-165, 2008.
DOI : 10.1016/j.bcmd.2007.07.017

D. Gale, S. Harten, C. Reid, E. Tuddenham, and P. Maxwell, Autosomal dominant erythrocytosis and pulmonary arterial hypertension associated with an activating HIF2?? mutation, Blood, vol.112, issue.3, pp.919-940, 2008.
DOI : 10.1182/blood-2008-04-153718

M. Martini, L. Teofili, T. Cenci, F. Giona, L. Torti et al., A novel heterozygous HIF2AM535I mutation reinforces the role of oxygen sensing pathway disturbances in the pathogenesis of familial erythrocytosis, Haematologica, vol.93, issue.7, pp.1068-71, 2008.
DOI : 10.3324/haematol.13210

M. Percy, P. Beer, G. Campbell, A. Dekker, A. Green et al., Novel exon 12 mutations in the HIF2A gene associated with erythrocytosis, Blood, vol.111, issue.11, pp.5400-5402, 2008.
DOI : 10.1182/blood-2008-02-137703

M. Percy, P. Furlow, P. Beer, T. Lappin, M. Mcmullin et al., A novel erythrocytosis-associated PHD2 mutation suggests the location of a HIF binding groove, Blood, vol.110, issue.6, pp.2193-2199, 2007.
DOI : 10.1182/blood-2007-04-084434

M. Percy, P. Furlow, G. Lucas, X. Li, T. Lappin et al., Gene in Familial Erythrocytosis, New England Journal of Medicine, vol.358, issue.2, pp.162-170, 2008.
DOI : 10.1056/NEJMoa073123

M. Percy, Q. Zhao, A. Flores, C. Harrison, T. Lappin et al., A family with erythrocytosis establishes a role for prolyl hydroxylase domain protein 2 in oxygen homeostasis, Proceedings of the National Academy of Sciences, vol.103, issue.3, pp.654-663, 2006.
DOI : 10.1073/pnas.0508423103

R. Van-wijk, S. Sutherland, V. Wesel, A. Huizinga, E. Percy et al., Erythrocytosis associated with a novel missense mutation in the HIF2A gene, Haematologica, vol.95, issue.5, pp.829-861, 2010.
DOI : 10.3324/haematol.2009.017582

C. Ladroue, R. Carcenac, M. Leporrier, S. Gad, L. Hello et al., Mutation and Congenital Erythrocytosis with Paraganglioma, New England Journal of Medicine, vol.359, issue.25, pp.2685-92, 2008.
DOI : 10.1056/NEJMoa0806277

F. Dayan, D. Roux, M. Brahimi-horn, J. Pouyssegur, and N. Mazure, The Oxygen Sensor Factor-Inhibiting Hypoxia-Inducible Factor-1 Controls Expression of Distinct Genes through the Bifunctional Transcriptional Character of Hypoxia-Inducible Factor-1??, Cancer Research, vol.66, issue.7, pp.3688-98, 2006.
DOI : 10.1158/0008-5472.CAN-05-4564
URL : https://hal.archives-ouvertes.fr/hal-00321066

J. Koditz, J. Nesper, M. Wottawa, D. Stiehl, G. Camenisch et al., Oxygen-dependent ATF-4 stability is mediated by the PHD3 oxygen sensor, Blood, vol.110, issue.10, pp.3610-3617, 2007.
DOI : 10.1182/blood-2007-06-094441

M. Ohh, C. Park, M. Ivan, M. Hoffman, T. Kim et al., Ubiquitination of hypoxia-inducible factor requires direct binding to the beta-domain of the von Hippel-Lindau protein, Nat Cell Biol, vol.2, issue.7, pp.423-430, 2000.

J. Huang, Q. Zhao, S. Mooney, and F. Lee, Sequence Determinants in Hypoxia-inducible Factor-1?? for Hydroxylation by the Prolyl Hydroxylases PHD1, PHD2, and PHD3, Journal of Biological Chemistry, vol.277, issue.42, pp.39792-800, 2002.
DOI : 10.1074/jbc.M206955200

M. Mcdonough, V. Li, E. Flashman, R. Chowdhury, C. Mohr et al., Cellular oxygen sensing: Crystal structure of hypoxia-inducible factor prolyl hydroxylase (PHD2), Proceedings of the National Academy of Sciences, vol.103, issue.26, pp.9814-9823, 2006.
DOI : 10.1073/pnas.0601283103

A. Steinhoff, F. Pientka, S. Mockel, A. Kettelhake, E. Hartmann et al., Cellular oxygen sensing: Importins and exportins are mediators of intracellular localisation of prolyl-4-hydroxylases PHD1 and PHD2, Biochemical and Biophysical Research Communications, vol.387, issue.4, pp.705-716, 2009.
DOI : 10.1016/j.bbrc.2009.07.090

S. Frede, P. Freitag, L. Geuting, R. Konietzny, and J. Fandrey, Oxygen-regulated expression of the erythropoietin gene in the human renal cell line REPC, Blood, vol.117, issue.18, pp.4905-4919, 2011.
DOI : 10.1182/blood-2010-07-298083

P. Dahia, K. Ross, M. Wright, C. Hayashida, S. Santagata et al., A HIF1alpha regulatory loop links hypoxia and mitochondrial signals in pheochromocytomas, PLoS Genet, vol.1, issue.1, pp.72-80, 2005.

G. Eisenhofer, T. Huynh, K. Pacak, F. Brouwers, M. Walther et al., Distinct gene expression profiles in norepinephrine- and epinephrine-producing hereditary and sporadic pheochromocytomas: activation of hypoxia-driven angiogenic pathways in von Hippel-Lindau syndrome, Endocrine Related Cancer, vol.11, issue.4, pp.897-911, 2004.
DOI : 10.1677/erc.1.00838

J. Favier, J. Briere, N. Burnichon, J. Riviere, L. Vescovo et al., The Warburg Effect Is Genetically Determined in Inherited Pheochromocytomas, PLoS ONE, vol.4, issue.9, p.7094, 2009.
DOI : 10.1371/journal.pone.0007094.t003

P. Pollard, M. El-bahrawy, R. Poulsom, G. Elia, P. Killick et al., Mutations, The Journal of Clinical Endocrinology & Metabolism, vol.91, issue.11, pp.4593-4601, 2006.
DOI : 10.1210/jc.2006-0920
URL : https://hal.archives-ouvertes.fr/hal-00652407

J. Mecinovic, R. Chowdhury, E. Flashman, and C. Schofield, Use of mass spectrometry to probe the nucleophilicity of cysteinyl residues of prolyl hydroxylase domain 2, Analytical Biochemistry, vol.393, issue.2, pp.215-236, 2009.
DOI : 10.1016/j.ab.2009.06.029

J. Mecinovic, R. Chowdhury, B. Lienard, E. Flashman, M. Buck et al., ESI-MS Studies on Prolyl Hydroxylase Domain???2 Reveal a New Metal Binding Site, ChemMedChem, vol.100, issue.4, pp.569-72, 2008.
DOI : 10.1002/cmdc.200700233

K. Nytko, N. Maeda, P. Schlafli, P. Spielmann, R. Wenger et al., Vitamin C is dispensable for oxygen sensing in vivo, Blood, vol.117, issue.20, pp.5485-93, 2011.
DOI : 10.1182/blood-2010-09-307637

M. Bordoli, D. Stiehl, L. Borsig, G. Kristiansen, S. Hausladen et al., Prolyl-4-hydroxylase PHD2- and hypoxia-inducible factor 2-dependent regulation of amphiregulin contributes to breast tumorigenesis, Oncogene, vol.59, issue.5, pp.548-60, 2011.
DOI : 10.1016/j.ccr.2009.09.029

D. Chan, T. Kawahara, P. Sutphin, H. Chang, J. Chi et al., Tumor Vasculature Is Regulated by PHD2-Mediated Angiogenesis and Bone Marrow-Derived Cell Recruitment, Cancer Cell, vol.15, issue.6, pp.527-565, 2009.
DOI : 10.1016/j.ccr.2009.04.010

Z. Shao, Y. Zhang, and J. Powell-coffman, Two Distinct Roles for EGL-9 in the Regulation of HIF-1-Mediated Gene Expression in Caenorhabditis elegans, Genetics, vol.183, issue.3, pp.821-830, 2009.
DOI : 10.1534/genetics.109.107284

D. Astuti, C. Ricketts, R. Chowdhury, M. Mcdonough, D. Gentle et al., Mutation analysis of HIF prolyl hydroxylases (PHD/EGLN) in individuals with features of phaeochromocytoma and renal cell carcinoma susceptibility, Endocrine Related Cancer, vol.18, issue.1, pp.73-83, 2010.
DOI : 10.1677/ERC-10-0113