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Targeting of HIF-α to the von Hippel-Lindau Ubiquitylation Complex by O ₂ -Regulated Prolyl Hydroxylation

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Pyruvate Kinase M2 Is a PHD3-Stimulated Coactivator for Hypoxia-Inducible Factor 1

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HIF-1α binding to VHL is regulated by stimulus-sensitive proline hydroxylation

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Placental but Not Heart Defects Are Associated with Elevated Hypoxia-Inducible Factor α Levels in Mice Lacking Prolyl Hydroxylase Domain Protein 2

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Mucosal Protection by Hypoxia-Inducible Factor Prolyl Hydroxylase Inhibition

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Inhibition of Prolyl Hydroxylases Increases Erythropoietin Production in ESRD

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A family with erythrocytosis establishes a role for prolyl hydroxylase domain protein 2 in oxygen homeostasis

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Inhibition of oxygen sensors as a therapeutic strategy for ischaemic and inflammatory disease

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Somatic inactivation of the PHD2 prolyl hydroxylase causes polycythemia and congestive heart failure

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The HIF Signaling Pathway in Osteoblasts Directly Modulates Erythropoiesis through the Production of EPO

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Prolyl 4-hydroxylases, key enzymes in the synthesis of collagens and regulation of the response to hypoxia, and their roles as treatment targets

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Regulation of adult erythropoiesis by prolyl hydroxylase domain proteins

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Essential role for prolyl hydroxylase domain protein 2 in oxygen homeostasis of the adult vascular system

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Involvement of oxygen-sensing pathways in physiologic and pathologic erythropoiesis

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Effect of desferrioxamine and metals on the hydroxylases in the oxygen sensing pathway

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Oxygen-dependent ATF-4 stability is mediated by the PHD3 oxygen sensor

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Abnormal Sympathoadrenal Development and Systemic Hypotension in PHD3−/− Mice

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HIF prolyl hydroxylase inhibition results in endogenous erythropoietin induction, erythrocytosis, and modest fetal hemoglobin expression in rhesus macaques

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Hypoxia-inducible factor-2α-expressing interstitial fibroblasts are the only renal cells that express erythropoietin under hypoxia-inducible factor stabilization

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Overexpression of PH-4, a novel putative proline 4-hydroxylase, modulates activity of hypoxia-inducible transcription factors

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Reactivation of hepatic EPO synthesis in mice after PHD loss

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Hepatic hypoxia-inducible factor-2 down-regulates hepcidin expression in mice through an erythropoietin-mediated increase in erythropoiesis

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Oxygen-Regulated β 2 -Adrenergic Receptor Hydroxylation by EGLN3 and Ubiquitylation by pVHL

Xie, L., Xiao, K., Whalen, E. J., Forrester, M. T., Freeman, R. S., Fong, G., Gygi, S. P., Lefkowitz, R. J., & Stamler, J. S. (2009) · Science Signaling · doi:10.1126/scisignal.2000444 · Cited by 148 · see citing works

Donor treatment with a PHD-inhibitor activating HIFs prevents graft injury and prolongs survival in an allogenic kidney transplant model

Bernhardt, W. M., Göttmann, U., Doyon, F., Buchholz, B., Câmpean, V., Schödel, J., Reisenbuechler, A., Klaus, S. J., Arend, M., Flippin, L. A., et al. (2009) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0903978106 · Cited by 145 · see citing works

An Endoplasmic Reticulum Transmembrane Prolyl 4-Hydroxylase Is Induced by Hypoxia and Acts on Hypoxia-inducible Factor α

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Control of Cyclin D1 and Breast Tumorigenesis by the EglN2 Prolyl Hydroxylase

Zhang, Q. H., Gu, J., Li, L., Liu, J., Luo, B., Cheung, H.-W., Boehm, J. S., Ni, M., Geisen, C., Root, D. E., et al. (2009) · Cancer Cell · doi:10.1016/j.ccr.2009.09.029 · Cited by 137 · see citing works

Hearts of Hypoxia-inducible Factor Prolyl 4-Hydroxylase-2 Hypomorphic Mice Show Protection against Acute Ischemia-Reperfusion Injury

Hyvärinen, J., Hassinen, I. E., Sormunen, R. T., Mäki, J. M., Kivirikko, K. I., Koivunen, P., & Myllyharju, J. (2010) · Journal of Biological Chemistry · doi:10.1074/jbc.m109.084855 · Cited by 107 · see citing works

The glial cell response is an essential component of hypoxia-induced erythropoiesis in mice

Weidemann, A., Kerdiles, Y. M., Knaup, K. X., Rafie, C. A., Boutin, A. T., Stockmann, C., Takeda, N., Scadeng, M., Shih, A. Y., Haase, V. H., et al. (2009) · Journal of Clinical Investigation · doi:10.1172/jci39378 · Cited by 100 · see citing works

Activation of hypoxia-inducible factors ameliorates hypoxic distal tubular injury in the isolated perfused rat kidney

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Pharmacological Characterization of 1-(5-Chloro-6-(trifluoromethoxy)-1H-benzoimidazol-2-yl)-1H-pyrazole-4-carboxylic Acid (JNJ-42041935), a Potent and Selective Hypoxia-Inducible Factor Prolyl Hydroxylase Inhibitor

Barrett, T. D., Palomino, H. L., Brondstetter, T. I., Kanelakis, K. C., Wu, X., Haug, P. V., Yan, W., Young, A., Hua, H., Hart, J. C., et al. (2011) · Molecular Pharmacology · doi:10.1124/mol.110.070508 · Cited by 78 · see citing works

HIF prolyl hydroxylase inhibitors for anemia

Muchnik, E., & Kaplan, J. (2011) · Expert Opinion on Investigational Drugs · doi:10.1517/13543784.2011.566861 · Cited by 76 · see citing works

Non-hypoxic Stabilization of Hypoxia-Inducible Factor Alpha (HIF-α): Relevance in Neural Progenitor/Stem Cells

Milošević, J., Adler, I., Manaenko, A., Schwarz, S. C., Walkinshaw, G., Arend, M., Flippin, L. A., Storch, A., & Schwarz, J. (2009) · Neurotoxicity Research · doi:10.1007/s12640-009-9043-z · Cited by 74 · see citing works

Hypoxia. 5. Hypoxia and hematopoiesis

Yoon, D., Ponka, P., & Prchal, J. T. (2011) · American Journal of Physiology-Cell Physiology · doi:10.1152/ajpcell.00044.2011 · Cited by 61 · see citing works

Hepcidin: A Critical Regulator of Iron Metabolism during Hypoxia

Hintze, K. J., & McClung, J. P. (2011) · Advances in Hematology · doi:10.1155/2011/510304 · Cited by 58 · see citing works

Distinct deregulation of the hypoxia inducible factor by PHD2 mutants identified in germline DNA of patients with polycythemia

Ladroue, C., Hoogewijs, D., Gad, S., Carcenac, R., Storti, F., Barrois, M., Gimenez-Roqueplo, A.-P., Leporrier, M., Casadevall, N., Hermine, O., et al. (2012) · Haematologica · doi:10.3324/haematol.2011.044644 · Cited by 55 · see citing works

HIF Prolyl 4-Hydroxylases and their Potential as Drug Targets

Myllyharju, J. (2009) · Current Pharmaceutical Design · doi:10.2174/138161209789649457 · Cited by 54 · see citing works

Deficiency of a Transmembrane Prolyl 4-Hydroxylase in the Zebrafish Leads to Basement Membrane Defects and Compromised Kidney Function

Hyvärinen, J., Parikka, M., Sormunen, R. T., Rämet, M., Tryggvason, K., Kivirikko, K. I., Myllyharju, J., & Koivunen, P. (2010) · Journal of Biological Chemistry · doi:10.1074/jbc.m110.145904 · Cited by 44 · see citing works

Isolated erythrocytosis: study of 67 patients and identification of three novel germ-line mutations in the prolyl hydroxylase domain protein 2 (PHD2) gene

Albiero, E., Ruggeri, M., Fortuna, S., Finotto, S., Bernardi, M., Madeo, D., & Rodeghiero, F. (2012) · Haematologica · doi:10.3324/haematol.2010.039545 · Cited by 28 · see citing works

Short-term effects of pharmacologic HIF stabilization on vasoactive and cytotrophic factors in developing mouse brain

Schneider, C., Krischke, G., Keller, S., Walkinshaw, G., Arend, M., Rascher, W., Gassmann, M., & Trollmann, R. (2009) · Brain Research · doi:10.1016/j.brainres.2009.05.023 · Cited by 18 · see citing works

253 Evaluation of FG-4592, a Novel Oral Hypoxia-Inducible Factor Prolyl Hydroxylase Inhibitor, to Treat Anemia in Hemodialysis Patients

Provenzano, R., Goodkin, D. A., Klaus, S. J., Linde, P. G., Kazazi, F., Lee, T. T., Neff, T. B., & Yu, P. (2011) · American Journal of Kidney Diseases · doi:10.1053/j.ajkd.2011.02.256 · Cited by 11 · see citing works

Regulation of erythropoiesis by hypoxia-inducible factors

Haase, V. H. (2013) · Blood Reviews · doi:10.1016/j.blre.2012.12.003 · Cited by 787 · see citing works

Anaemia in kidney disease: harnessing hypoxia responses for therapy

Koury, M. J., & Haase, V. H. (2015) · Nature Reviews Nephrology · doi:10.1038/nrneph.2015.82 · Cited by 313 · see citing works

Mechanisms of hypoxia signalling: new implications for nephrology

Schödel, J., & Ratcliffe, P. J. (2019) · Nature Reviews Nephrology · doi:10.1038/s41581-019-0182-z · Cited by 309 · see citing works

HIF prolyl hydroxylase inhibitors for the treatment of renal anaemia and beyond

Maxwell, P. H., & Eckardt, K. (2015) · Nature Reviews Nephrology · doi:10.1038/nrneph.2015.193 · Cited by 286 · see citing works

The EGLN-HIF O 2 -Sensing System: Multiple Inputs and Feedbacks

Ivan, M., & Kaelin, W. G. (2017) · Molecular Cell · doi:10.1016/j.molcel.2017.06.002 · Cited by 273 · see citing works

Mimicking Hypoxia to Treat Anemia: HIF-Stabilizer BAY 85-3934 (Molidustat) Stimulates Erythropoietin Production without Hypertensive Effects

Flamme, I., Oehme, F., Ellinghaus, P., Jeske, M., Keldenich, J., & Thuß, U. (2014) · PLoS ONE · doi:10.1371/journal.pone.0111838 · Cited by 170 · see citing works

EGLN1 Inhibition and Rerouting of α-Ketoglutarate Suffice for Remote Ischemic Protection

Olenchock, B. A., Moslehi, J. J., Baik, A. H., Davidson, S. M., Williams, J., Gibson, W. J., Chakraborty, A. A., Pierce, K. A., Miller, C. M., Hanse, E. A., et al. (2016) · Cell · doi:10.1016/j.cell.2016.02.006 · Cited by 141 · see citing works

IRP1 regulates erythropoiesis and systemic iron homeostasis by controlling HIF2α mRNA translation

Wilkinson, N., & Pantopoulos, K. (2013) · Blood · doi:10.1182/blood-2013-03-492454 · Cited by 133 · see citing works

Adaptation of iron requirement to hypoxic conditions at high altitude

Gassmann, M., & Muckenthaler, M. U. (2015) · Journal of Applied Physiology · doi:10.1152/japplphysiol.00248.2015 · Cited by 123 · see citing works

Prolyl 4‐hydroxylases, master regulators of the hypoxia response

Myllyharju, J. (2013) · Acta Physiologica · doi:10.1111/apha.12096 · Cited by 118 · see citing works

HIF Prolyl 4-Hydroxylase-2 Inhibition Improves Glucose and Lipid Metabolism and Protects Against Obesity and Metabolic Dysfunction

Rahtu‐Korpela, L., Karsikas, S., Hörkkö, S., Sequeiros, R. T. B., Lammentausta, E., Mäkelä, K. A., Herzig, K., Walkinshaw, G., Kivirikko, K. I., Myllyharju, J., et al. (2014) · Diabetes · doi:10.2337/db14-0472 · Cited by 118 · see citing works

Inflammation, fibrosis, and modulation of the process by mesenchymal stem/stromal cells

Prockop, D. J. (2016) · Matrix Biology · doi:10.1016/j.matbio.2016.01.010 · Cited by 95 · see citing works

Hypoxia-Inducible Factor Prolyl 4-Hydroxylase-2 Inhibition Protects Against Development of Atherosclerosis

Rahtu‐Korpela, L., Määttä, J., Dimova, E. Y., Hörkkö, S., Gylling, H., Walkinshaw, G., Hakkola, J., Kivirikko, K. I., Myllyharju, J., Serpi, R., et al. (2016) · Arteriosclerosis Thrombosis and Vascular Biology · doi:10.1161/atvbaha.115.307136 · Cited by 90 · see citing works

Increased EPO Levels Are Associated With Bone Loss in Mice Lacking PHD2 in EPO-Producing Cells

Rauner, M., Franke, K., Murray, M., Singh, R. P., Hiram‐Bab, S., Platzbecker, U., Gassmann, M., Socolovsky, M., Neumann, D., Gabet, Y., et al. (2016) · Journal of Bone and Mineral Research · doi:10.1002/jbmr.2857 · Cited by 68 · see citing works

Renal epithelium regulates erythropoiesis via HIF-dependent suppression of erythropoietin

Farsijani, N., Liu, Q., Kobayashi, H., Davidoff, O., Sha, F., Fandrey, J., Ikizler, T. A., O'Connor, P. M., & Haase, V. H. (2016) · Journal of Clinical Investigation · doi:10.1172/jci74997 · Cited by 67 · see citing works

Renal anemia: from incurable to curable

Sato, Y., & Yanagita, M. (2013) · American Journal of Physiology-Renal Physiology · doi:10.1152/ajprenal.00233.2013 · Cited by 65 · see citing works

Targeting EPO and EPO receptor pathways in anemia and dysregulated erythropoiesis

Rainville, N., Jachimowicz, E., & Wojchowski, D. M. (2015) · Expert Opinion on Therapeutic Targets · doi:10.1517/14728222.2016.1090975 · Cited by 43 · see citing works

Targeting the Hypoxia-Sensing Pathway in Clinical Hematology

Forristal, C. E., & Lévesque, J.-P. (2013) · Stem Cells Translational Medicine · doi:10.5966/sctm.2013-0134 · Cited by 41 · see citing works

Hepcidin as a therapeutic target for anemia and inflammation associated with chronic kidney disease

Małyszko, J., Małyszko, J. S., & Matuszkiewicz‐Rowińska, J. (2019) · Expert Opinion on Therapeutic Targets · doi:10.1080/14728222.2019.1599358 · Cited by 39 · see citing works

Hypoxia-inducible factor prolyl-4-hydroxylase-1 is a convergent point in the reciprocal negative regulation of NF-κB and p53 signaling pathways

Ullah, K., Rosendahl, A.-H., Izzi, V., Bergmann, U., Pihlajaniemi, T. A., Mäki, J. M., & Myllyharju, J. (2017) · Scientific Reports · doi:10.1038/s41598-017-17376-0 · Cited by 34 · see citing works

Genome-wide identification of genes probably relevant to the adaptation of schizothoracins (Teleostei: Cypriniformes) to the uplift of the Qinghai-Tibet Plateau

Chi, W., Ma, X., Niu, J., & Zou, M. (2017) · BMC Genomics · doi:10.1186/s12864-017-3703-9 · Cited by 31 · see citing works

Hematological, Hepatic, and Retinal Phenotypes in Mice Deficient for Prolyl Hydroxylase Domain Proteins in the Liver

Duan, L., Takeda, K., & Fong, G. (2014) · American Journal Of Pathology · doi:10.1016/j.ajpath.2013.12.014 · Cited by 30 · see citing works

Hypoxia-inducible factor prolyl 4-hydroxylase inhibition in cardiometabolic diseases

Koivunen, P., Serpi, R., & Dimova, E. Y. (2016) · Pharmacological Research · doi:10.1016/j.phrs.2016.11.003 · Cited by 31 · see citing works

Biallelic loss-of-function P4HTM gene variants cause hypotonia, hypoventilation, intellectual disability, dysautonomia, epilepsy, and eye abnormalities (HIDEA syndrome)

Rahikkala, E., Myllykoski, M., Hinttala, R., Vieira, P., Nayebzadeh, N., Weiss, S., Plomp, A. S., Bittner, R. E., Kurki, M., Kuismin, O., et al. (2019) · Genetics in Medicine · doi:10.1038/s41436-019-0503-4 · Cited by 27 · see citing works

Drugs activating hypoxia-inducible factors correct erythropoiesis and hepcidin levels via renal EPO induction in mice

Nakai, T., Iwamura, Y., Kato, K., Hirano, I., Matsumoto, Y., Tomioka, Y., Yamamoto, M. P., & Suzuki, N. (2023) · Blood Advances · doi:10.1182/bloodadvances.2023009798 · Cited by 25 · see citing works

Hypoxia-Inducible Factor Stabilizers: a New Avenue for Reducing BP While Helping Hemoglobin?

Yousaf, F., & Spinowitz, B. (2016) · Current Hypertension Reports · doi:10.1007/s11906-016-0629-6 · Cited by 23 · see citing works

Lack of P4H-TM in mice results in age-related retinal and renal alterations

Leinonen, H., Rossi, M., Salo, A. M., Tiainen, P., Hyvärinen, J., Pitkänen, M., Sormunen, R. T., Miinalainen, I., Zhang, C., Soininen, R., et al. (2016) · Human Molecular Genetics · doi:10.1093/hmg/ddw228 · Cited by 22 · see citing works

HIF prolyl hydroxylase inhibitor FG-4497 enhances mouse hematopoietic stem cell mobilization via VEGFR2/KDR

Bisht, K., Brunck, M. E. G., Matsumoto, T., McGirr, C., Nowlan, B., Fleming, W., Keech, T., Magor, G. W., Perkins, A. C., Davies, J. M., et al. (2019) · Blood Advances · doi:10.1182/bloodadvances.2018017566 · Cited by 21 · see citing works

Null mutation in P4h-tm leads to decreased fear and anxiety and increased social behavior in mice

Leinonen, H., Koivisto, H., Lipponen, H.-R., Matilainen, A., Salo, A. M., Dimova, E. Y., Hämäläinen, E., Stavén, S., Miettinen, P., Myllyharju, J., et al. (2019) · Neuropharmacology · doi:10.1016/j.neuropharm.2019.04.023 · Cited by 17 · see citing works

Biallelic Mutations in P4HTM Cause Syndromic Obesity

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Structure-based drug design for hypoxia-inducible factor prolyl-hydroxylase inhibitors and its therapeutic potential for the treatment of erythropoiesis-stimulating agent-resistant anemia: raising expectations for exploratory clinical trials

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Exploring links between 2‐oxoglutarate‐dependent oxygenases and Alzheimer's disease

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A Pro‐Regenerative Supramolecular Prodrug Protects Against and Repairs Colon Damage in Experimental Colitis

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FG-4497: a new target for acute respiratory distress syndrome?

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Current and Novel Drugs for Management of Anemia

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Short title: P4H-TM AND ERYTHROPOIESIS

(2016)

An Endoplasmic Reticulum Transmembrane Prolyl 4-Hydroxylase Is Induced by Hypoxia and Acts on Hypoxia-inducible Factor α

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Inhibition of Prolyl Hydroxylases Increases Erythropoietin Production in ESRD

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