• The role of mTOR signaling network and growth suppressor TSC2 in pulmonary vascular cell reprograming, PA remodeling and right ventricular dysfunction in pulmonary arterial hypertension. (ucdavis.edu)
  • As endothelial dysfunction is a key feature of PAH, and as endothelial progenitor cells (EPC) may contribute to vascular repair in PAH, we suspected that prostacyclin therapy might enhance EPC numbers and functions. (harvard.edu)
  • Human endothelial progenitor cells (hEPCs) are adult stem cells, located in the bone marrow and peripheral blood. (intechopen.com)
  • Different phenotypes and subtypes of endothelial progenitor cells (EPCs), such as early and late EPCs, have been described according to their functionality. (intechopen.com)
  • Endothelial progenitor cells (EPCs) contribute to neovascularization and play an important role in the development of these diseases. (hindawi.com)
  • The bone-marrow-derived endothelial progenitor cells (EPCs) provide an alternative source of endothelial cells (ECs) that contributes to neovessel formation in endothelium structure [ 4 , 5 ]. (hindawi.com)
  • Pulmonary endothelial dysfunction and inflammation, triggered by shear stress and hypoxia, constitute the hallmarks of pulmonary vasculopathy by promoting endothelial and smooth muscle cells proliferation, vasoconstriction, and thrombosis. (mjrheum.org)
  • Pulmonary arterial hypertension (PAH) is characterized by dysregulated cell proliferation and infiltration of activated inflammatory cells leading to progressive narrowing and obliteration of the distal pulmonary arteries. (nih.gov)
  • Mitochondrial fission and a metabolic switch from oxidative phosphorylation to glycolysis are key features of vascular pathology in pulmonary arterial hypertension (PAH) and are associated with exuberant endothelial proliferation and apoptosis . (bvsalud.org)
  • Dysfunction in a myriad of overlapping signalling pathways can promote endothelial cell (EC) proliferation and differentiation, smooth muscle cell (SMC) proliferation, migration and vasoconstriction, pericyte proliferation, migration and differentiation, endothelial-to-mesenchymal transition, immune cell infiltration and extracellular matrix (ECM) remodelling in the pulmonary artery. (ersjournals.com)
  • The role of HIPPO signaling cassette in pulmonary vascular cell hyper-proliferation, development and progression of pulmonary hypertension. (ucdavis.edu)
  • 4 In addition, vascular endothelial growth factor (VEGF) is an angiogenic factor that regulates endothelial survival, proliferation, differentiation, and vascular permeability, and has been shown to be an important candidate for cancer therapy as it is upregulated in many tumors. (beckman.com)
  • These alterations can influence endothelial dysfunction, excessive vasoconstriction, and abnormal cell proliferation of the vascular wall. (ajmc.com)
  • Risk factors include a family history, prior pulmonary embolism (blood clots in the lungs), HIV/AIDS, sickle cell disease, cocaine use, chronic obstructive pulmonary disease, sleep apnea, living at high altitudes, and problems with the mitral valve. (wikipedia.org)
  • To stress the importance of considering pulmonary complications in sickle cell disease (SCD), researchers compiled an overview on the state of research and available treatment options for patients with SCD associated with pulmonary hypertension. (ajmc.com)
  • Although rare, pulmonary hypertension (PH) can be a serious complication from sickle cell disease (SCD). (ajmc.com)
  • This study is first to demonstrate the key role of endothelial intracellular chloride channels in the regulation of mitochondrial structure, biogenesis, and metabolic reprogramming in expression of the PAH phenotype . (bvsalud.org)
  • Association of deep vein thrombosis type with clinical phenotype of chronic thromboembolic pulmonary hypertension. (cdc.gov)
  • Impaired natural killer cell phenotype and function in idiopathic and heritable pulmonary arterial hypertension. (cdc.gov)
  • Background Blood flow is closely related to function, but currently, the relationship of right ventricular (RV) blood flow components with RV function and hemodynamics in patients with chronic thromboembolic pulmonary hypertension (CTEPH) remains unclear. (researchgate.net)
  • High prevalence of dysfibrinogenemia among patients with chronic thromboembolic pulmonary hypertension. (cdc.gov)
  • The pathogenesis of pulmonary arterial hypertension (WHO Group I) involves the narrowing of blood vessels connected to and within the lungs. (wikipedia.org)
  • Diverse cellular processes and signalling pathways contribute to the pathogenesis of pulmonary arterial hypertension (PAH). (ersjournals.com)
  • Asthma and pulmonary arterial hypertension: do they share a key mechanism of pathogenesis? (ers-education.org)
  • These mechanistic pathways are critical for the pathogenesis of coronary heart disease, hypertension, and atrial fibrillation, all of which are etiological risk factors for end-stage cardiovascular disease. (medscape.com)
  • Background Pulmonary vasodilators in general and prostacyclin therapy in particular, have markedly improved the outcome of patients with pulmonary arterial hypertension (PAH). (harvard.edu)
  • Background Pulmonary hypertension (PH) is a common complication of COPD, associated with increased mortality and morbidity. (ersjournals.com)
  • Methods We quantified CD34+ cells, CFU-Hill and ECFC (endothelial colony forming cells) in peripheral blood from children with idiopathic PAH (n = 27) or PAH secondary to congenital heart disease (n = 52). (harvard.edu)
  • While research was predominantly focused on pulmonary vasculature, the investigation of peripheral endothelial damage in different vascular beds has attracted the interest over the last years. (mjrheum.org)
  • As a result, effective non-invasive methods that can assess the endothelial function and the architectural integrity have been utilized for the evaluation of pulmonary and peripheral vasculature. (mjrheum.org)
  • Non-invasive plethysmography, pulmonary flow reserve, nailfold videocapillaroscopy, near-infrared spectroscopy, and imaging techniques such as magnetic resonance angiography and perfusion imaging coupled by a number of biomarkers can be used for the assessment of peripheral vascular function in PAH individuals. (mjrheum.org)
  • Imaging to diagnose pulmonary thromboembolic disease, peripheral pulmonary artery stenosis, pulmonary vein stenosis, pulmonary veno-occlusive disease (PVOD), and parenchymal lung disease should be performed at the time of diagnosis. (medscape.com)
  • Pulmonary hypertension (PH or PHTN) is a condition of increased blood pressure in the arteries of the lungs. (wikipedia.org)
  • A 1973 World Health Organization meeting was the first attempt to classify pulmonary hypertension by its cause, and a distinction was made between primary PH (resulting from a disease of the pulmonary arteries) and secondary PH (resulting secondary to other, non-vascular causes). (wikipedia.org)
  • Image shows accumulation of specialized immune system cells around tiny arteries in lungs of mice with pulmonary hypertension. (scitechdaily.com)
  • First author Abdul Sheikh and senior author Daniel Greif had previously identified specialized cells in the smooth muscle of small arteries in the lungs of mice with pulmonary hypertension (PH), as well as in human cells. (scitechdaily.com)
  • Researchers in EMBL's Zaugg group have studied the causes of pulmonary arterial hypertension (PAH), a rare disease that causes high blood pressure in the arteries of the lungs. (embl.org)
  • The results suggested that the rewiring of the regulatory network in the cells surrounding the blood vessels, called endothelial cells, makes it possible for them to differentiate to become smooth muscle cells, which then contribute to the obstruction of the lung arteries. (embl.org)
  • By showing that epigenetic changes may prime endothelial cells to differentiate into smooth muscle cells, which would then obstruct arteries, it also raises the question of what exactly triggers this huge rearrangement. (embl.org)
  • Endothelial cells (ECs) form the endothelium, a layer of single-cell thickness that coats the interior wall of all blood vessels including arteries, capillaries, and veins. (beckman.com)
  • PAH is characterized by progressive, obliterative remodeling of pulmonary arterioles, pre-capillary vessel loss, right heart failure and death. (nature.com)
  • Pulmonary arterial hypertension (PAH) is a lethal vasculopathy characterized by pathogenic remodeling of pulmonary arterioles leading to increased pulmonary pressures, right ventricular hypertrophy, and heart failure. (biomedcentral.com)
  • Fibrinogen Aa Thr312Ala polymorphism specifically contributes to chronic thromboembolic pulmonary hypertension by increasing fibrin resistance. (cdc.gov)
  • These guidelines are endorsed by the International Society for Heart and Lung Transplantation, and provide the current framework for understanding and treatment of pulmonary hypertension. (wikipedia.org)
  • The focus of the Pulmonary Vascular Biology Lab, led by Dr. Jason Elinoff, is the interplay between lung vascular endothelium and immune effector cells and their contributions to the development and progression of pulmonary arterial hypertension (PAH). (nih.gov)
  • Pulmonary arterial hypertension (PAH) is a progressive disease characterized by lung endothelial cell dysfunction and vascular remodeling. (surrey.ac.uk)
  • Researchers at EMBL investigate the epigenetic changes in a rare lung disease, pulmonary arterial hypertension. (embl.org)
  • Cigarette smoke (CS) is well known to be a risk factor for pulmonary diseases including chronic obstructive pulmonary disease (COPD), lung cancer, asthma, pulmonary hypertension, and vascular diseases such as atherosclerosis [ 1 ]. (hindawi.com)
  • Using innovative cell culture systems, lung disease models, and human biosamples we investigate the impacts of environmental exposures on smooth muscle, epithelial, endothelial and immune cells. (umanitoba.ca)
  • The standard treatment for CTEPH is and should remain pulmonary endarterectomy, a surgery that clears clots and scar material from the blood vessels of the lung. (drugtopics.com)
  • This possibility is supported by the finding of microthrombi in the pulmonary vascular bed, which are noted at the time of lung biopsy, autopsy, or in explanted lungs at the time of lung transplantation. (medscape.com)
  • After endocardial cushion formation, the endothelial mesenchymal transformation (EMT), which are specified endothelial cells, differentiate and migrate into the cardiac jelly. (medscape.com)
  • Histologic assessment of patients with systemic sclerosis-associated PAH and the hypoxia/SU5416 mouse model identified the presence von Willebrand factor/α-smooth muscle actin-positive endothelial cells in up to 5% of pulmonary vessels. (surrey.ac.uk)
  • The minimal hemodynamic change that defines a positive response to AVT for children should be considered as a ≥20% decrease in PAP and pulmonary vascular resistance (PVR)/systemic vascular resistance (SVR) without a decrease in cardiac output. (medscape.com)
  • OSA is a cause of systemic hypertension (HTN) and is associated with an increased incidence of stroke, heart failure (HF), atrial fibrillation (AF), and coronary heart disease (CHD). (medscape.com)
  • With further study, the researchers aim to pinpoint a specific target for therapies to treat pulmonary hypertension earlier and more effectively. (scitechdaily.com)
  • The pathobiology of pulmonary vascular disease (PVD) and PAH is complex, multifactorial and driven by inflammation and metabolic dysfunction 1 . (nature.com)
  • GATA6 transcriptional factor as a regulator of pulmonary vascular cell-cell communications, BMP signaling and inflammation in pulmonary hypertension. (ucdavis.edu)
  • Oxidative stress and inflammation in the vascular wall are essential mechanisms of atherosclerosis and vascular dysfunctions associated with risk factors such as metabolic diseases, aging, hypertension, etc. (frontiersin.org)
  • Research focuses on inflammation, tissue repair and developmental biology in the context of asthma, chronic obstructive pulmonary disease, pulmonary hypertension, and congenital diaphragmatic hernia. (umanitoba.ca)
  • The illustration depicts the multietiological risk factors for sleep apnea and its downstream consequences, which include increased sympathetic nerve activity, metabolic dysregulation, inflammation, oxidative stress, vascular endothelial dysfunction, and intermittent hypoxia. (medscape.com)
  • Previously, we identified inducible nitric oxide synthase (iNOS) as an essential enzyme for development and reversal of smoke-induced PH and emphysema, and showed that iNOS expression in bone-marrow-derived cells drives pulmonary vascular remodelling, but not parenchymal destruction. (ersjournals.com)
  • Pulmonary hypertension is associated with endothelial dysfunction, impaired synthesis of nitric oxide and insufficient stimulation of the NO-sGC-cGMP pathway. (globalrph.com)
  • Berner M, Beghetti M, Saphr-Schopfer I, Oberhansli I, Friedli: Inhaled Nitric Oxide to test the vasodilator capacity of the pulmonary vascular bed in children with long-standing pulmonary hypertension and congenital disease. (medigraphic.com)
  • Wessel D, Adatia I, Giglia T, Thompson J, Kuilik T: Use of inhaled nitric oxide and acetylcholine in the evaluation of pulmonary hypertension and endothelial function after cardiopulmonary bypass. (medigraphic.com)
  • Curran R, Mavroudis C, Backer C, Backer C, Sautel M, Zales V, et al: Inhaled nitric oxide for children with congenital heart disease and pulmonary hypertension. (medigraphic.com)
  • Evaluation of pulmonary arterial endothelial cells and smooth muscle cells revealed that miR-130/301 targeted PPARγ with distinct consequences. (jci.org)
  • Current work includes in vitro profiling of human pulmonary artery endothelial cells with heterogeneous PAH-associated molecular defects in order to investigate molecular mechanisms that can exploited therapeutically. (nih.gov)
  • Pathological overexpression of CLIC proteins induces mitochondrial fragmentation, inhibits mitochondrial cristae formation and induces metabolic shift towards glycolysis in human pulmonary artery endothelial cells , consistent with changes observed in patient -derived cells . (bvsalud.org)
  • We determined the presence of EndoMT in the pulmonary vasculature in vivo and the functional effects on pulmonary artery endothelial cells (PAECs) undergoing EndoMT in vitro. (surrey.ac.uk)
  • Pulmonary edema in patients with pulmonary veno-occlusive disease. (globalrph.com)
  • Prior to joining Surrey, her research centred around pulmonary hypertension and connective tissue diseases. (surrey.ac.uk)
  • The nanoparticle we developed is a powerful new delivery system for genome editing in vascular endothelial cells, and could be used to treat many diseases, including acute respiratory distress syndrome from severe COVID-19," said senior author Dr. Zhao from Lurie Children's. (eurekalert.org)
  • Endothelial dysfunction is at the root of many diseases, such as coronary artery disease, stroke, bronchopulmonary dysplasia and pulmonary artery hypertension. (eurekalert.org)
  • Cigarette smoke is a major public health problem associated with multitude of diseases, including pulmonary and vascular diseases. (hindawi.com)
  • Hypoimmune induced pluripotent stem cell-derived cell therapeutics treat cardiovascular and pulmonary diseases in immunocompetent allogeneic mice. (ucsf.edu)
  • Has also been considered a risk factor for various isolated diseases, including chronic obstructive pulmonary disease (COPD), different types of cancer, hemodynamic alterations, vascular disease, infectious diseases, among others. (who.int)
  • Endothelial CLIC4 excision and mitofusin 2 supplementation have protective effects in human PAH cells and pre-clinical PAH. (bvsalud.org)
  • Single-cell RNA sequencing profiling of mouse endothelial cells in response to pulmonary arterial hypertension. (nih.gov)
  • The majority of VWF is synthesized by endothelial cells (ECs) and stored in Weibel-Palade bodies (WPB). (imperial.ac.uk)
  • During embryogenesis endothelial cells exhibit substantial plasticity that contribute to cardiac development by undergoing endothelial-to-mesenchymal transition (EndoMT). (surrey.ac.uk)
  • Induced EndoMT cells exhibited up-regulation of mesenchymal markers, including collagen type I and α-smooth muscle actin, and a reduction in endothelial cell and junctional proteins, including von Willebrand factor, CD31, occludin, and vascular endothelial-cadherin. (surrey.ac.uk)
  • The lab of Youyang Zhao, PhD , from Stanley Manne Children's Research Institute at Ann & Robert H. Lurie Children's Hospital of Chicago developed a unique nanoparticle to deliver genome editing technology, including CRISPR/Cas9, to endothelial cells, which are cells that line blood vessel walls. (eurekalert.org)
  • This is the first time that vascular endothelial cells could be reached for genome editing, since the usual way to deliver CRISPR/Cas9 - through a virus - does not work for this cell type. (eurekalert.org)
  • Dr. Zhao explained that genome editing in endothelial cells could even treat cancers by cutting off the blood supply to the tumor or blocking cancer metastasis. (eurekalert.org)
  • In endothelial cells, miR-130/301 modulated apelin-miR-424/503-FGF2 signaling, while in smooth muscle cells, miR-130/301 modulated STAT3-miR-204 signaling to promote PH-associated phenotypes. (jci.org)
  • During early development, the myocardial cushion begins as a matrix of endothelial cells and an outer mitochondrial layer separated by cardiac jelly. (medscape.com)
  • Endothelial cells (ECs) line the inner surfaces of the blood vessels and lymphatic vessels in the body. (bmbreports.org)
  • These cells can be differentiated into mature endothelial cells, which are involved in processes of angiogenesis and vessel regeneration. (intechopen.com)
  • Thus, it has been shown that early EPCs release cytokines that promote tissue regeneration and neovasculogenesis, whereas late EPC and endothelial colony forming cells (ECFCs) contribute to the formation of blood vessels and stimulate tube formation. (intechopen.com)
  • In addition, the number and the migratory activity of these cells are inversely correlated with risk factors such as hypertension, hypercholesterolemia, diabetes, and metabolic syndrome. (intechopen.com)
  • Evidence has been provided that activation of the vascular endothelial cells in the presence of the risk factors promotes oxidative stress and vascular inflammatory responses, leading to acceleration of atherosclerotic vascular disease. (frontiersin.org)
  • Primordial endothelial cells specialize into tissue-specific phenotypes of arterial, venous, hemogenic, and lymphatic subtypes with distinct functions for vascular development. (beckman.com)
  • Endothelial cells form a semi-permeable barrier between the blood and surrounding tissues within all blood vessels in the body. (beckman.com)
  • Coughing up of blood may occur in some patients, particularly those with specific subtypes of pulmonary hypertension such as heritable pulmonary arterial hypertension, Eisenmenger syndrome and chronic thromboembolic pulmonary hypertension. (wikipedia.org)
  • While current therapeutics reduce pulmonary arterial pressure and increase time to death or transplant, median survival remains only 5-7 years from diagnosis. (nih.gov)
  • The diagnosis is confirmed regardless of the pulmonary arterial pressure, as long as it is accompanied by a right-to-left shunt and absence of congenital heart disease. (medscape.com)
  • The remaining cases can be associated with precapillary arterial PH that can result from the obstructive remodeling of the pulmonary vascular bed. (ajmc.com)
  • Pulmonary venous hypertension typically presents with shortness of breath while lying flat or sleeping (orthopnea or paroxysmal nocturnal dyspnea), while pulmonary arterial hypertension (PAH) typically does not. (wikipedia.org)
  • Unlike selective pulmonary vasodilators alone, approaches directed at inflammatory vascular remodeling have the potential to arrest or even reverse the disease. (nih.gov)
  • Treatment is with pulmonary vasodilators and diuretics. (msdmanuals.com)
  • The endothelial to mesenchymal transition (EndMT) is a newly recognized, fundamental biological process involved in development and tissue regeneration, as well as pathological processes such as the complications of diabetes, fibrosis and pulmonary arterial hypertension. (bmbreports.org)
  • Several histologic subtypes are associated with pulmonary arteriopathy in IPAH, one of which involves in situ thrombosis. (medscape.com)
  • 2011. Dietary nitrate attenuates oxidative stress, prevents cardiac and renal injuries, and reduces blood pressure in salt-induced hypertension. (cdc.gov)
  • These disordered breathing events are associated with a profile of perturbations that include intermittent hypoxia, oxidative stress, sympathetic activation, and endothelial dysfunction, all of which are critical mediators of cardiovascular disease. (medscape.com)
  • Mineralocorticoid receptor antagonist treatment of established pulmonary arterial hypertension improves interventricular dependence in the SU5416-hypoxia rat model. (nih.gov)
  • Developmental Programming of Pulmonary Hypertension by Isolated Chronic Prenatal Hypoxia. (ed.ac.uk)
  • A large study called the Second Natural History Study of Congenital Heart Defects analyzed the treatment, quality of life, echocardiography findings, complications, exercise responses, and predisposition to endocarditis with regards to cardiac valvular disease, and pulmonary stenosis was found to be the most benign valvular lesion. (medscape.com)
  • The American Heart Association and American Thoracic Society released guidelines on pediatric pulmonary hypertension. (medscape.com)
  • In clinical arena, Dr. Cornfield is a Pediatrician with an active practice in both Pediatric Pulmonary Medicine and Pediatric Critical Care Medicine. (stanford.edu)
  • As of 2022[update] there was no cure for pulmonary hypertension, although research to find a cure is ongoing. (wikipedia.org)
  • Other typical signs of pulmonary hypertension include an accentuated pulmonary component of the second heart sound, a right ventricular third heart sound, and parasternal heave indicating a hypertrophied right ventricle. (wikipedia.org)
  • Here we report application of human umbilical cord mesenchymal stem cell (HUCMSC)-derived therapy for pulmonary arterial hypertension (PAH). (nature.com)
  • ECs have the capacity to undergo a dynamic cellular phenotypic switching, termed the endothelial to mesenchymal transition (EndMT), in response to local environmental cues throughout the vascular system. (bmbreports.org)
  • Background At present, the alterations in molecular markers and signaling pathways in chronic thromboembolic pulmonary hypertension (CTEPH) remain unclear. (researchgate.net)
  • Persistent/recurrent Chronic Thromboembolic Pulmonary Hypertension (CTEPH) (WHO Group 4) after surgical treatment or inoperable CTEPH to improve exercise capacity and WHO functional class. (globalrph.com)
  • FDA has approved riociguat (Adempas, Bayer HealthCare Pharmaceuticals) tablets for the treatment of adults with chronic thromboembolic pulmonary hypertension (CTEPH) after surgical treatment, adults with inoperable CTEPH, and adults with pulmonary arterial hypertension (PAH). (drugtopics.com)
  • The approval of Adempas equips physicians with a new treatment option for patients with PAH and CTEPH, two life-threatening forms of pulmonary hypertension," said Pamela A. Cyrus, MD, vice president and head, U.S. medical, Bayer HealthCare Pharmaceuticals. (drugtopics.com)
  • Development of the vascular disease pulmonary hypertension (PH) involves disparate molecular pathways that span multiple cell types. (jci.org)