ArticleArteriosclerosis, thrombosis, and vascular biology2017
Codependence of Bone Morphogenetic Protein Receptor 2 and Transforming Growth Factor-β in Elastic Fiber Assembly and Its Perturbation in Pulmonary Arterial Hypertension.
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Article in Arteriosclerosis, thrombosis, and vascular biology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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33citing papers in PubMed
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33 citing papers in PubMed, 46 citations in OpenAlex.
- Activin Signaling Inhibitors in Pulmonary Hypertension: A State-of-the-Art Review.Pulmonary circulation · 2026Review
- Case Report: A case of severe pulmonary hypertension combined with FBN1 mutation associated geleophysic dysplasia.Frontiers in pediatrics · 2025Article
- Proteomic Signatures of Right Ventricular Outcomes in Pulmonary Arterial Hypertension.Circulation. Heart failure · 2024Article
- Bioengineered vascular grafts with a pathogenicScience translational medicine · 2024Article
- A computational growth and remodeling framework for adaptive and maladaptive pulmonary arterial hemodynamics.Biomechanics and modeling in mechanobiology · 2023Article
- Elastin stabilization prevents impaired biomechanics in human pulmonary arteries and pulmonary hypertension in rats with left heart disease.Nature communications · 2023Article
- A Computational Growth and Remodeling Framework for Adaptive and Maladaptive Pulmonary Arterial Hemodynamics.bioRxiv : the preprint server for biology · 2023Article
- Elastic Fibers and F-Box and WD-40 Domain-Containing Protein 2 in Bovine Periosteum and Blood Vessels.Biomimetics (Basel, Switzerland) · 2022Article
- Single-cell transcriptomics reveals skewed cellular communication and phenotypic shift in pulmonary artery remodeling.JCI insight · 2022Article
- Compromised Cardiopulmonary Function in Fibulin-5 Deficient Mice.Journal of biomechanical engineering · 2022Article
- Development of vascular disease models to explore disease causation and pathomechanisms of rare vascular diseases.Seminars in immunopathology · 2022Review
- The Mechanobiology of Vascular Remodeling in the Aging Lung.Physiology (Bethesda, Md.) · 2022Review
- Observational
- Reduced neutrophil elastase inhibitor elafin and elevated transforming growth factor-βERJ open research · 2021Article
- Phenotypic diversity and metabolic specialization of renal endothelial cells.Nature reviews. Nephrology · 2021Review
- Human iPS Cell-derived Tissue Engineered Vascular Graft: Recent Advances and Future Directions.Stem cell reviews and reports · 2021Review
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- Effects of BMP-2 compound with fibrin on osteoporotic vertebral fracture healing in rats.Journal of musculoskeletal & neuronal interactions · 2021Article
- Xenogeneic-free generation of vascular smooth muscle cells from human induced pluripotent stem cells for vascular tissue engineering.Acta biomaterialia · 2021Article
- Mendelian randomization and experimental IUGR reveal the adverse effect of low birth weight on lung structure and function.Scientific reports · 2020Article
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Authors and funding
12 authors at 1 institution in 1 country.
Nancy F TojaisFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Aiqin CaoFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Ying-Ju LaiFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Lingli WangFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Pin-I ChenFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Miguel A Alejandre AlcazarFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Vinicio A de Jesus PerezFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Rachel K HopperFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Christopher J RhodesFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Matthew A BillFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Lynn Y SakaiFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.).
Marlene RabinovitchFrom the Department of Pediatrics (N.F.T., A.C., Y.-J.L., L.W., P.I.C., M.A.A.A., R.K.H., C.J.R., M.R.) and Department of Medicine (V.A.d.J.P., M.A.B.), the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA; and Shriners Hospital for Children, Oregon Health & Science University, Portland (L.Y.S.). marlener@stanford.edu.
Shriners Hospitals for Children - Portland · US
Funding
Michigan Institute for Clinical and Health Research (MICHR)UL1TR002240 · NCATS · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI LUMENG, JULIE C, MASHOUR, GEORGE ALEXANDER · 2017 to 2022
$54.9MMICHIGAN INSTITUTE FOR CLINICAL AND HEALTH RESEARCH (MICHR) (UL1): (BPCA)UL1RR024986 · NCRR · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI SHANLEY, THOMAS P. · 2007 to 2011
$51.2MPulmonary Hypertension in Genetically Modified MiceR01HL074186 · NHLBI · STANFORD UNIVERSITY · PI RABINOVITCH, MARLENE · 2004 to 2022
$10.4MPulmonary Hypertension Breakthrough InitiativeR24HL123767 · NHLBI · UNIVERSITY OF COLORADO DENVER · PI ALDRED, MICHEALA A, ERZURUM, SERPIL C. · 2014 to 2017
$10.1MAirway redox biochemistry as a deteriminant of asthma phenotype during adolescen*U10HL109250 · NHLBI · UNIVERSITY OF VIRGINIA · PI ERZURUM, SERPIL C., GASTON, BENJAMIN · 2011 to 2016
$4.2MNCATS NIH HHS UL1 TR002240NCRR NIH HHS UL1 RR024986NHLBI NIH HHS R01 HL074186NHLBI NIH HHS R24 HL123767NHLBI NIH HHS U10 HL109250
Abstract
objectiveWe determined in patients with pulmonary arterial (PA) hypertension (PAH) whether in addition to increased production of elastase by PA smooth muscle cells previously reported, PA elastic fibers are susceptible to degradation because of their abnormal assembly. APPROACH AND
resultsFibrillin-1 and elastin are the major components of elastic fibers, and fibrillin-1 binds bone morphogenetic proteins (BMPs) and the large latent complex of transforming growth factor-β1 (TGFβ1). Thus, we considered whether BMPs like TGFβ1 contribute to elastic fiber assembly and whether this process is perturbed in PAH particularly when the BMP receptor, BMPR2, is mutant. We also assessed whether in mice with
conclusionsDisrupting BMPR2 impairs TGFβ1- and BMP4-mediated elastic fiber assembly and is of pathophysiologic significance in PAH.
Indexed as
Vascular RemodelingAdipokinesAnimalsBone Morphogenetic Protein 4Bone Morphogenetic Protein Receptors, Type IBone Morphogenetic Protein Receptors, Type IICase-Control StudiesCells, CulturedDisease Models, AnimalElastic TissueElastinFamilial Primary Pulmonary HypertensionFibrillin-1FibroblastsGenetic Predisposition to DiseaseHumansAdipokinesBmpr1a protein, mouseBMPR2 protein, humanBmpr2 protein, mouseBone Morphogenetic Protein 4Bone Morphogenetic Protein Receptors, Type IBone Morphogenetic Protein Receptors, Type IIElastinFBN1 protein, humanFbn1 protein, mouseFibrillin-1Transforming Growth Factor betaBMPR2 receptorelastinfibrillin-1fibroblastshypertension, pulmonarysmooth muscle cellsTGF-beta-1
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