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Results: 26
BMP-9 induced endothelial cell tubule formation and inhibition of migration involves Smad1 driven endothelin-1 production.
John ES Park, Dongmin Shao, Paul D Upton, Patricia Desouza, Ian M Adcock, Rachel J Davies, Nicholas W Morrell, Mark JD Griffiths, Stephen J Wort
Jan 23, 2018
BACKGROUND: Bone morphogenetic proteins (BMPs) and their receptors, such as bone morphogenetic protein receptor (BMPR) II, have been implicated in a wide variety of disorders including pulmonary arterial hypertension (PAH)....
4PBA Restores Signaling of a Cysteine-substituted Mutant BMPR2 Receptor Found in Patients with Pulmonary Arterial Hypertension.
Benjamin J Dunmore, Xudong Yang, Alexi Crosby, Stephen Moore, Lu Long, Christopher Huang, Mark Southwood, Eric D Austin, Amer Rana, Paul D Upton, Nicholas W Morrell
Apr 06, 2020
Mutations in the gene encoding BMPR2 (bone morphogenetic protein type 2 receptor) are the major cause of heritable pulmonary arterial hypertension (PAH). Point mutations in the BMPR2 ligand-binding domain involving cysteine...
Hypertension Mutation pulmonary Treatment Animals Bone Morphogenetic Protein Receptors Type II Cell Proliferation Cells Cultured Cysteine Humans Mice Muscle Smooth Vascular Mutation Myocytes Smooth Muscle Organophosphorus Compounds pulmonary arterial hypertension Pulmonary Artery Signal Transduction Vascular Remodeling
Neutrophil-mediated IL-6 receptor trans-signaling and the risk of chronic obstructive pulmonary disease and asthma
N Farahi, E Paige, J Balla, E Prudence, RC Ferreira, M Southwood, SL Appleby, P Bakke, A Gulsvik, AA Litonjua, D Sparrow, EK Silverman, MH Cho, J Danesh, DS Paul, DF Freitag, ER Chilvers
Apr 28, 2017
Alleles chronic obstructive airway disease Pulmonary Artery Signal Transduction Endothelial Cells Lung Asthma Genes Homozygote monocyte chemoattractant protein-1 neutrophil interleukin 6 receptor Respiration Disorders Interleukin-6 extrinsic asthma in vitro study squamous intraepithelial lesion pmel17 tocilizumab biobanks
Selective enhancement of endothelial BMPR-II with BMP9 reverses pulmonary arterial hypertension.
Lu Long, Mark L Ormiston, Xudong Yang, Mark Southwood, Stefan Gräf, Rajiv D Machado, Matthias Mueller, Bernd Kinzel, Lai Ming Yung, Janine M Wilkinson, Stephen D Moore, Kylie M Drake, Micheala A Aldred, Paul B Yu, Paul D Upton, Nicholas W Morrell
Jan 23, 2018
Genetic evidence implicates the loss of bone morphogenetic protein type II receptor (BMPR-II) signaling in the endothelium as an initiating factor in pulmonary arterial hypertension (PAH). However, selective targeting of this...
aging Animals Apoptosis Bone Morphogenetic Protein Receptors Type II Cell Membrane Permeability Densitometry Endothelial Cells Gene Expression Profiling Gene Knock-In Techniques Growth Differentiation Factor 2 Heart Ventricles Humans Hypertension Pulmonary Immunoblotting JNK Mitogen-Activated Protein Kinases Male Mice Inbred C57BL Monocrotaline Phosphorylation Pulmonary Artery Rats Rats Sprague-Dawley Systole Transcription Genetic
CAMPHOR score
Michael Newnham, Katherine Bunclark, Nisha Abraham, Samantha Ali, Liliana Amaral-Almeida, John E Cannon, Natalie Doughty, Choo Ng, Anie Ponnaberanam, Karen Sheares, Nicola Speed, Dolores Taboada, Mark Toshner, Steven Tsui, David P Jenkins, Joanna Pepke-Zaba
May 13, 2020
BACKGROUND: Pulmonary endarterectomy (PEA) is the recommended treatment for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH). The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) score is an...
Transcript analysis reveals a specific HOX signature associated with positional identity of human endothelial cells.
Mark Toshner, Benjamin J Dunmore, Eoin F McKinney, Mark Southwood, Paola Caruso, Paul D Upton, John P Waters, Mark L Ormiston, Jeremy N Skepper, Gerard Nash, Amer A Rana, Nicholas W Morrell
Jun 14, 2018
The endothelial cell has a remarkable ability for sub-specialisation, adapted to the needs of a variety of vascular beds. The role of developmental programming versus the tissue contextual environment for this specialization is...
Adult Animals Cell Differentiation Cell Line Cluster Analysis Embryonic Stem Cells Endothelial Cells Fetus Gene Expression Profiling Gene Ontology Genes Homeobox Homeodomain Proteins Humans Lung Mice Neovascularization Physiologic Oligonucleotide Array Sequence Analysis Organ Specificity Phenotype Pulmonary Artery RNA Messenger Rats Real-Time Polymerase Chain Reaction Reproducibility of Results
Isolated large vessel pulmonary vasculitis as a cause of chronic obstruction of the pulmonary arteries.
Guy Hagan, Deepa Gopalan, Colin Church, Doris Rassl, Chetan Mukhtyar, Trevor Wistow, Chim Lang, Pasupathy Sivasothy, Susan Stewart, David Jayne, Karen Sheares, Steven Tsui, David P Jenkins, Joanna Pepke-Zaba
Dec 15, 2020
Isolated pulmonary artery involvement by large vessel vasculitis is rare. This case report describes two patients with large vessel pulmonary vasculitis initially thought to have chronic thromboembolic pulmonary hypertension who...
Approaches to treat pulmonary arterial hypertension by targeting BMPR2
Pulmonary arterial hypertension (PAH) is estimated to affect between 10 and 50 people per million worldwide. The lack of cure and devastating nature of the disease means that treatment is crucial to arrest rapid clinical...
Single-cell RNA sequencing profiling of mouse endothelial cells in response to pulmonary arterial hypertension.
Julie Rodor, Shiau Haln Chen, Jessica P Scanlon, João P Monteiro, Axelle Caudrillier, Sweta Sweta, Katherine Ross Stewart, Alena Shmakova, Ross Dobie, Beth EP Henderson, Kevin Stewart, Patrick WF Hadoke, Mark Southwood, Stephen D Moore, Paul D Upton, Nick W Morrell, Ziwen Li, Stephen Y Chan, Adam Handen, Robert Lafyatis, Laura PMH de Rooij, Neil C Henderson, Peter Carmeliet, Ana Mishel Spiroski, Mairi Brittan, Andrew H Baker
Oct 05, 2021
AIMS: Endothelial cell (EC) dysfunction drives the initiation and pathogenesis of pulmonary arterial hypertension (PAH). We aimed to characterize EC dynamics in PAH at single-cell resolution. METHODS AND RESULTS: We carried out...
A diagnostic miRNA signature for pulmonary arterial hypertension using a consensus machine learning approach.
Niamh Errington, James Iremonger, Josephine A Pickworth, Sokratis Kariotis, Christopher J Rhodes, Alexander MK Rothman, Robin Condliffe, Charles A Elliot, David G Kiely, Luke S Howard, John Wharton, AA Roger Thompson, Nicholas W Morrell, Martin R Wilkins, Dennis Wang, Allan Lawrie
Jul 30, 2021
BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare but life shortening disease, the diagnosis of which is often delayed, and requires an invasive right heart catheterisation. Identifying diagnostic biomarkers may...
MicroRNA-140-5p and SMURF1 regulate pulmonary arterial hypertension.
Alexander MK Rothman, Nadine D Arnold, Josephine A Pickworth, James Iremonger, Loredana Ciuclan, Robert MH Allen, Sabine Guth-Gundel, Mark Southwood, Nicholas W Morrell, Matthew Thomas, Sheila E Francis, David J Rowlands, Allan Lawrie
Jun 27, 2018
Loss of the growth-suppressive effects of bone morphogenetic protein (BMP) signaling has been demonstrated to promote pulmonary arterial endothelial cell dysfunction and induce pulmonary arterial smooth muscle cell (PASMC)...
Pulmonary arterial stiffening in COPD and its implications for right ventricular remodelling.
Jonathan R Weir-McCall, Patrick Sk Liu-Shiu-Cheong, Allan D Struthers, Brian J Lipworth, J Graeme Houston
Oct 03, 2019
BACKGROUND: Pulmonary pulse wave velocity (PWV) allows the non-invasive measurement of pulmonary arterial stiffening, but has not previously been assessed in COPD. The aim of the current study was to assess PWV in COPD and its...
Heart Ventricles Magnetic Resonance Imaging Pulmonary arteries Pulmonary Disease Chronic Obstructive Pulse-wave analysis Aged Female Heart Ventricles Humans Magnetic Resonance Imaging Male Middle Aged Pulmonary Artery Pulmonary Disease Chronic Obstructive Pulse Wave Analysis Respiratory Function Tests Ventricular Remodeling
TNFα drives pulmonary arterial hypertension by suppressing the BMP type-II receptor and altering NOTCH signalling
LA Hurst, BJ Dunmore, L Long, A Crosby, R Al-Lamki, J Deighton, M Southwood, X Yang, MZ Nikolic, B Herrera, GJ Inman, JR Bradley, AA Rana, PD Upton, NW Morrell
Feb 20, 2017
ADAM10 Protein Animals Bone Morphogenetic Protein 6 Bone Morphogenetic Protein Receptors Type II Familial Primary Pulmonary Hypertension Female Humans Male Mice Mice Inbred C57BL Myocytes Smooth Muscle Pulmonary Artery Rats Receptor Notch2 Receptor Notch3 Signal Transduction Tumor Necrosis Factor-alpha
Highlights from the International Chronic Thromboembolic Pulmonary Hypertension Congress 2021.
Gérald Simonneau, Elie Fadel, Anton Vonk Noordegraaf, Mark Toshner, Irene M Lang, Frederikus A Klok, Micheal C McInnis, Nicholas Screaton, Michael M Madani, Guillermo Martinez, Kiran Salaunkey, David P Jenkins, Hiromi Matsubara, Philippe Brénot, Marius M Hoeper, Hossein A Ghofrani, Xavier Jaïs, Christoph B Wiedenroth, Stefan Guth, Nick H Kim, Joanna Pepke-Zaba, Marion Delcroix, Eckhard Mayer
Mar 13, 2023
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. It is caused by persistent obstruction of pulmonary arteries by chronic organised fibrotic clots, despite adequate...
A therapeutic antibody targeting osteoprotegerin attenuates severe experimental pulmonary arterial hypertension.
Nadine D Arnold, Josephine A Pickworth, Laura E West, Sarah Dawson, Joana A Carvalho, Helen Casbolt, Adam T Braithwaite, James Iremonger, Lewis Renshall, Volker Germaschewski, Matthew McCourt, Philip Bland-Ward, Hager Kowash, Abdul G Hameed, Alexander MK Rothman, Maria G Frid, AA Roger Thompson, Holly R Evans, Mark Southwood, Nicholas W Morrell, David C Crossman, Moira Kb Whyte, Kurt R Stenmark, Christopher M Newman, David G Kiely, Sheila E Francis, Allan Lawrie
Jan 20, 2020
Pulmonary arterial hypertension (PAH) is a rare but fatal disease. Current treatments increase life expectancy but have limited impact on the progressive pulmonary vascular remodelling that drives PAH. Osteoprotegerin (OPG) is...
The lysosomal inhibitor, chloroquine, increases cell surface BMPR-II levels and restores BMP9 signalling in endothelial cells harbouring BMPR-II mutations.
Benjamin J Dunmore, Kylie M Drake, Paul D Upton, Mark R Toshner, Micheala A Aldred, Nicholas W Morrell
Apr 06, 2020
Pulmonary arterial hypertension (PAH) is characterized by dysregulated pulmonary artery endothelial cell (PAEC) proliferation, apoptosis and permeability. Loss-of-function mutations in the bone morphogenetic protein receptor...
Bone Morphogenetic Protein Receptors Type II Cell Line Cell Membrane Cells Cultured Chloroquine Endothelial Cells Familial Primary Pulmonary Hypertension Growth Differentiation Factor 2 Growth Differentiation Factors Humans Hypertension Pulmonary Lysosomes Mutation Pulmonary Artery RNA Small Interfering Signal Transduction Smad Proteins Transcription Genetic
Molecular genetic framework underlying pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is a rare, progressive disorder typified by occlusion of the pulmonary arterioles owing to endothelial dysfunction and uncontrolled proliferation of pulmonary artery smooth muscle cells and...
A Potential Role for Exosomal Translationally Controlled Tumor Protein Export in Vascular Remodeling in Pulmonary Arterial Hypertension.
Elisabet Ferrer, Benjamin J Dunmore, Dhiya Hassan, Mark L Ormiston, Stephen Moore, John Deighton, Lu Long, Xu Dong Yang, Duncan J Stewart, Nicholas W Morrell
Aug 09, 2018
Endothelium Exosomes Hypertension pulmonary remodeling Animals Apoptosis Biomarkers Tumor Bone Morphogenetic Protein Receptors Type II Cell Movement Cell Proliferation Cell Shape Disease Models Animal Endothelial Cells Exosomes Humans Hypertension Pulmonary Lentivirus Lung Male Monocrotaline Mutation Myocytes Smooth Muscle Protein Transport Pulmonary Artery Rats Sprague-Dawley Tumor Protein Translationally-Controlled 1 Vascular Remodeling
Angiostrongylus chabaudi in felids
Alessio Giannelli, Zvezedlina Kirkova, Francesca Abramo, Maria Stefania Latrofa, Bronwyn Campbell, Nicola Zizzo, Cinzia Cantacessi, Filipe Dantas-Torres, Domenico Otranto
Oct 28, 2016
Role of BRCA1-associated protein (BRAP) variant in childhood pulmonary arterial hypertension
Ayako Chida-Nagai, Masaki Shintani, Hiroki Sato, Tomotaka Nakayama, Masaki Nii, Hiroyuki Akagawa, Toru Furukawa, Amer Rana, Yoshiyuki Furutani, Kei Inai, Shigeaki Nonoyama, Toshio Nakanishi
Mar 13, 2019
Letter by Morrell et al Regarding Article, "Selective BMP-9 Inhibition Partially Protects Against Experimental Pulmonary Hypertension".
We read with interest the article by Tu et al1 demonstrating that genetic loss of Bmp9 (bone morphogenetic protein 9) protects mice from hypoxia-induced pulmonary hypertension and that inhibition of BMP9 with anti-BMP9 or the...
Measurement of extravascular lung water to diagnose severe reperfusion lung injury following pulmonary endarterectomy
The measurement of extravascular lung water is a relatively new technology which has not yet been well validated as a clinically useful tool. We studied its utility in patients undergoing pulmonary endarterectomy as they...
Spontaneous pulmonary hypertension in genetic mouse models of natural killer cell deficiency.
Matthew T Rätsep, Stephen D Moore, Salema Jafri, Melissa Mitchell, Hugh JM Brady, Ofer Mandelboim, Mark Southwood, Nicholas W Morrell, Francesco Colucci, Mark L Ormiston
Nov 21, 2018
Natural killer (NK) cells are cytotoxic innate lymphoid cells with an established role in the regulation of vascular structure in pregnancy and cancer. Impaired NK cell function has been identified in patients with pulmonary...
Th17 angiopoietin-2 Interleukin-23 natural killer cells pulmonary hypertension Animals Basic-Leucine Zipper Transcription Factors Disease Models Animal Endothelial Cells Humans Hypertension Pulmonary Killer Cells Natural Lung Mice Natural Cytotoxicity Triggering Receptor 1 Pulmonary Artery Vascular Remodeling
Balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension
Stephen P Hoole, John G Coghlan, John E Cannon, Dolores Taboada, Mark Toshner, Karen Sheares, Andrew John Fletcher, Guillermo Martinez, Alessandro Ruggiero, Nicholas Screaton, David Jenkins, Joanna Pepke-Zaba
May 06, 2020
OBJECTIVE: Inoperable chronic thromboembolic pulmonary hypertension (CTEPH) managed medically has a poor prognosis. Balloon pulmonary angioplasty (BPA) offers a new treatment for inoperable patients. The national BPA service for...
Balloon pulmonary angioplasty Chronic thromboembolic pulmonary hypertension Right heart function Aged Angioplasty Balloon Arterial Pressure Chronic Disease Exercise Tolerance Female Humans Hypertension Pulmonary Male Middle Aged Pulmonary Artery pulmonary embolism Recovery of Function Time Factors Treatment Outcome United Kingdom Vascular Resistance Ventricular Function Right Ventricular Remodeling
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