Search Results - "MORRELL, Nicholas W"

Molecular genetic framework underlying pulmonary arterial hypertension by Southgate, Laura, Machado, Rajiv D., Gräf, Stefan, Morrell, Nicholas W.

“…Pulmonary arterial hypertension (PAH) is a rare, progressive disorder typified by occlusion of the pulmonary arterioles owing to endothelial dysfunction and…”
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Relevant Issues in the Pathology and Pathobiology of Pulmonary Hypertension by Tuder, Rubin M., MD, Archer, Stephen L., MD, Dorfmüller, Peter, MD, PhD, Erzurum, Serpil C., MD, Guignabert, Christophe, PhD, Michelakis, Evangelos, MD, Rabinovitch, Marlene, MD, Schermuly, Ralph, PhD, Stenmark, Kurt R., MD, Morrell, Nicholas W., MD

“…Knowledge of the pathobiology of pulmonary hypertension (PH) continues to accelerate. However, fundamental gaps remain in our understanding of the underlying…”
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Genetics and genomics of pulmonary arterial hypertension by Morrell, Nicholas W, Aldred, Micheala A, Chung, Wendy K, Elliott, C Gregory, Nichols, William C, Soubrier, Florent, Trembath, Richard C, Loyd, James E

“…Since 2000 there have been major advances in our understanding of the genetic and genomics of pulmonary arterial hypertension (PAH), although there remains…”
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2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT by Hansmann, Georg, Koestenberger, Martin, Alastalo, Tero-Pekka, Apitz, Christian, Austin, Eric D., Bonnet, Damien, Budts, Werner, D'Alto, Michele, Gatzoulis, Michael A., Hasan, Babar S., Kozlik-Feldmann, Rainer, Kumar, R. Krishna, Lammers, Astrid E., Latus, Heiner, Michel-Behnke, Ina, Miera, Oliver, Morrell, Nicholas W., Pieles, Guido, Quandt, Daniel, Sallmon, Hannes, Schranz, Dietmar, Tran-Lundmark, Karin, Tulloh, Robert M.R., Warnecke, Gregor, Wåhlander, Håkan, Weber, Sven C., Zartner, Peter

“…The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative…”
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Metabolic and Proliferative State of Vascular Adventitial Fibroblasts in Pulmonary Hypertension Is Regulated Through a MicroRNA-124/PTBP1 (Polypyrimidine Tract Binding Protein 1)/Pyruvate Kinase Muscle Axis by Zhang, Hui, Wang, Daren, Li, Min, Plecitá-Hlavatá, Lydie, D'Alessandro, Angelo, Tauber, Jan, Riddle, Suzette, Kumar, Sushil, Flockton, Amanda, McKeon, B Alexandre, Frid, Maria G, Reisz, Julie A, Caruso, Paola, El Kasmi, Karim C, Ježek, Petr, Morrell, Nicholas W, Hu, Cheng-Jun, Stenmark, Kurt R

“…An emerging metabolic theory of pulmonary hypertension (PH) suggests that cellular and mitochondrial metabolic dysfunction underlies the pathology of this…”
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Elabela/Toddler Is an Endogenous Agonist of the Apelin APJ Receptor in the Adult Cardiovascular System, and Exogenous Administration of the Peptide Compensates for the Downregulation of Its Expression in Pulmonary Arterial Hypertension by Yang, Peiran, Read, Cai, Kuc, Rhoda E, Buonincontri, Guido, Southwood, Mark, Torella, Rubben, Upton, Paul D, Crosby, Alexi, Sawiak, Stephen J, Carpenter, T Adrian, Glen, Robert C, Morrell, Nicholas W, Maguire, Janet J, Davenport, Anthony P

“…BACKGROUND:Elabela/toddler (ELA) is a critical cardiac developmental peptide that acts through the G-protein–coupled apelin receptor, despite lack of sequence…”
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Identification of MicroRNA-124 as a Major Regulator of Enhanced Endothelial Cell Glycolysis in Pulmonary Arterial Hypertension via PTBP1 (Polypyrimidine Tract Binding Protein) and Pyruvate Kinase M2 by Caruso, Paola, Dunmore, Benjamin J, Schlosser, Kenny, Schoors, Sandra, Dos Santos, Claudia, Perez-Iratxeta, Carol, Lavoie, Jessie R, Zhang, Hui, Long, Lu, Flockton, Amanda R, Frid, Maria G, Upton, Paul D, D'Alessandro, Angelo, Hadinnapola, Charaka, Kiskin, Fedir N, Taha, Mohamad, Hurst, Liam A, Ormiston, Mark L, Hata, Akiko, Stenmark, Kurt R, Carmeliet, Peter, Stewart, Duncan J, Morrell, Nicholas W

“…Pulmonary arterial hypertension (PAH) is characterized by abnormal growth and enhanced glycolysis of pulmonary artery endothelial cells. However, the…”
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MicroRNA-143 Activation Regulates Smooth Muscle and Endothelial Cell Crosstalk in Pulmonary Arterial Hypertension by Deng, Lin, Blanco, Francisco J, Stevens, Hannah, Lu, Ruifang, Caudrillier, Axelle, McBride, Martin, McClure, John D, Grant, Jenny, Thomas, Matthew, Frid, Maria, Stenmark, Kurt, White, Kevin, Seto, Anita G, Morrell, Nicholas W, Bradshaw, Angela C, MacLean, Margaret R, Baker, Andrew H

“…RATIONALE:The pathogenesis of pulmonary arterial hypertension (PAH) remains unclear. The 4 microRNAs representing the miR-143 and miR-145 stem loops are…”
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TNFα drives pulmonary arterial hypertension by suppressing the BMP type-II receptor and altering NOTCH signalling by Hurst, Liam A., Dunmore, Benjamin J., Long, Lu, Crosby, Alexi, Al-Lamki, Rafia, Deighton, John, Southwood, Mark, Yang, Xudong, Nikolic, Marko Z., Herrera, Blanca, Inman, Gareth J., Bradley, John R., Rana, Amer A., Upton, Paul D., Morrell, Nicholas W.

“…Heterozygous germ-line mutations in the bone morphogenetic protein type-II receptor (BMPR-II) gene underlie heritable pulmonary arterial hypertension (HPAH)…”
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Chloroquine Prevents Progression of Experimental Pulmonary Hypertension via Inhibition of Autophagy and Lysosomal Bone Morphogenetic Protein Type II Receptor Degradation by Long, Lu, Yang, Xudong, Southwood, Mark, Lu, Junyu, Marciniak, Stefan J, Dunmore, Benjamin J, Morrell, Nicholas W

“…RATIONALE:Pulmonary arterial hypertension (PAH) is characterized by excessive proliferation and apoptosis resistance in pulmonary artery smooth muscle cells…”
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Role of soluble endoglin in BMP9 signaling by Lawera, Aleksandra, Tong, Zhen, Thorikay, Midory, Redgrave, Rachael E., Cai, Jie, van Dinther, Maarten, Morrell, Nicholas W., Afink, Gijs B., Charnock-Jones, D. Stephen, Arthur, Helen M., Dijke, Peter ten, Li, Wei

“…Endoglin (ENG) is a coreceptor of the transforming growth factor-β (TGFβ) family signaling complex, which is highly expressed on endothelial cells and plays a…”
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Regulation of the ALK1 ligands, BMP9 and BMP10 by Li, Wei, Salmon, Richard M, Jiang, He, Morrell, Nicholas W

“…Bone morphogenetic protein (BMP)9 and BMP10 are high affinity ligands for activin receptor-like kinase 1 (ALK1), a type I BMP receptor mainly expressed on…”
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Plasma Metabolomics Implicates Modified Transfer RNAs and Altered Bioenergetics in the Outcomes of Pulmonary Arterial Hypertension by Rhodes, Christopher J, Ghataorhe, Pavandeep, Wharton, John, Rue-Albrecht, Kevin C, Hadinnapola, Charaka, Watson, Geoffrey, Bleda, Marta, Haimel, Matthias, Coghlan, Gerry, Corris, Paul A, Howard, Luke S, Kiely, David G, Peacock, Andrew J, Pepke-Zaba, Joanna, Toshner, Mark R, Wort, S John, Gibbs, J Simon R, Lawrie, Allan, Gräf, Stefan, Morrell, Nicholas W, Wilkins, Martin R

“…Pulmonary arterial hypertension (PAH) is a heterogeneous disorder with high mortality. We conducted a comprehensive study of plasma metabolites using…”
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Elevated levels of inflammatory cytokines predict survival in idiopathic and familial pulmonary arterial hypertension by Soon, Elaine, Holmes, Alan M, Treacy, Carmen M, Doughty, Natalie J, Southgate, Laura, Machado, Rajiv D, Trembath, Richard C, Jennings, Simon, Barker, Lucy, Nicklin, Paul, Walker, Christoph, Budd, David C, Pepke-Zaba, Joanna, Morrell, Nicholas W

“…Inflammation is a feature of pulmonary arterial hypertension (PAH), and increased circulating levels of cytokines are reported in patients with PAH. However,…”
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Inflammation, Growth Factors, and Pulmonary Vascular Remodeling by Hassoun, Paul M., MD, Mouthon, Luc, MD, PhD, Barberà, Joan A., MD, Eddahibi, Saadia, PhD, Flores, Sonia C., PhD, Grimminger, Friedrich, MD, PhD, Jones, Peter Lloyd, PhD, Maitland, Michael L., MD, PhD, Michelakis, Evangelos D., MD, Morrell, Nicholas W., MA, MD, Newman, John H., MD, Rabinovitch, Marlene, MD, Schermuly, Ralph, PhD, Stenmark, Kurt R., MD, Voelkel, Norbert F., MD, Yuan, Jason X.-J., MD, PhD, Humbert, Marc, MD, PhD

“…Inflammatory processes are prominent in various types of human and experimental pulmonary hypertension (PH) and are increasingly recognized as major pathogenic…”
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BMP9 Mutations Cause a Vascular-Anomaly Syndrome with Phenotypic Overlap with Hereditary Hemorrhagic Telangiectasia by Wooderchak-Donahue, Whitney L., McDonald, Jamie, O’Fallon, Brendan, Upton, Paul D., Li, Wei, Roman, Beth L., Young, Sarah, Plant, Parker, Fülöp, Gyula T., Langa, Carmen, Morrell, Nicholas W., Botella, Luisa M., Bernabeu, Carmelo, Stevenson, David A., Runo, James R., Bayrak-Toydemir, Pinar

“…Hereditary hemorrhagic telangiectasia (HHT), the most common inherited vascular disorder, is caused by mutations in genes involved in the transforming growth…”
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Molecular basis of ALK1-mediated signalling by BMP9/BMP10 and their prodomain-bound forms by Salmon, Richard M., Guo, Jingxu, Wood, Jennifer H., Tong, Zhen, Beech, John S., Lawera, Aleksandra, Yu, Minmin, Grainger, David J., Reckless, Jill, Morrell, Nicholas W., Li, Wei

“…Activin receptor-like kinase 1 (ALK1)-mediated endothelial cell signalling in response to bone morphogenetic protein 9 (BMP9) and BMP10 is of significant…”
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Pulmonary Arterial Hypertension: A Current Perspective on Established and Emerging Molecular Genetic Defects by Machado, Rajiv D., Southgate, Laura, Eichstaedt, Christina A., Aldred, Micheala A., Austin, Eric D., Best, D. Hunter, Chung, Wendy K., Benjamin, Nicola, Elliott, C. Gregory, Eyries, Mélanie, Fischer, Christine, Gräf, Stefan, Hinderhofer, Katrin, Humbert, Marc, Keiles, Steven B., Loyd, James E., Morrell, Nicholas W., Newman, John H., Soubrier, Florent, Trembath, Richard C., Viales, Rebecca Rodríguez, Grünig, Ekkehard

“…ABSTRACT Pulmonary arterial hypertension (PAH) is an often fatal disorder resulting from several causes including heterogeneous genetic defects. While…”
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Myocardin regulates vascular smooth muscle cell inflammatory activation and disease by Ackers-Johnson, Matthew, Talasila, Amarnath, Sage, Andrew P, Long, Xiaochun, Bot, Ilze, Morrell, Nicholas W, Bennett, Martin R, Miano, Joseph M, Sinha, Sanjay

“…Atherosclerosis, the cause of 50% of deaths in westernized societies, is widely regarded as a chronic vascular inflammatory disease. Vascular smooth muscle…”
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BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis by Evans, Jonathan D W, MBChB, Girerd, Barbara, PhD, Montani, David, MD, Wang, Xiao-Jian, PhD, Galiè, Nazzareno, MD, Austin, Eric D, MD, Elliott, Greg, Prof, Asano, Koichiro, MD, Grünig, Ekkehard, Prof, Yan, Yi, MD, Jing, Zhi-Cheng, Prof, Manes, Alessandra, MD, Palazzini, Massimiliano, MD, Wheeler, Lisa A, Nakayama, Ikue, MD, Satoh, Toru, Prof, Eichstaedt, Christina, PhD, Hinderhofer, Katrin, PhD, Wolf, Matthias, Rosenzweig, Erika B, MD, Chung, Wendy K, MD, Soubrier, Florent, Prof, Simonneau, Gérald, Prof, Sitbon, Olivier, Prof, Gräf, Stefan, PhD, Kaptoge, Stephen, PhD, Di Angelantonio, Emanuele, MD, Humbert, Marc, Prof, Morrell, Nicholas W, Prof

“…Summary Background Mutations in the gene encoding the bone morphogenetic protein receptor type II ( BMPR2 ) are the commonest genetic cause of pulmonary…”
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