ArticleCell stem cell2017
Patient-Specific iPSC-Derived Endothelial Cells Uncover Pathways that Protect against Pulmonary Hypertension in BMPR2 Mutation Carriers.
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Article in Cell stem cell, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 147 papers.
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147 citing papers in PubMed, 206 citations in OpenAlex.
- Argininosuccinate Lyase Deficiency Causes an Endothelial-Dependent Form of Hypertension.American journal of human genetics · 2018Trial
- Human iPSC-Derived Vascularised Lung Organoids for Modelling COPD and Pulmonary Hypertension.Cell proliferation · 2026Article
- Emerging role of DYRK1A as a target in cardiovascular diseases (Review).Molecular medicine reports · 2026Review
- Human stem cell models in cerebral cavernous malformations.Stem cell research & therapy · 2026Review
- Vascularized Cardiac Tissue Engineering: From Advances in Biofabrication to Translational Applications.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Review
- Rebalancing NTRK2 isoforms promotes vascular regeneration in bronchopulmonary dysplasia.Cell stem cell · 2026Article
- Induced pluripotent stem cells from sporadic pulmonary veno-occlusive disease exhibit altered endothelial commitment and stress-related transcriptional programs.Frontiers in cell and developmental biology · 2026Article
- Pathophysiology of androgen-associated endothelial cell dysfunction in phenotype A polycystic ovarian syndrome revealed by iPSCs modeling.Frontiers in endocrinology · 2026Article
- MTHFR variant links homocysteine metabolism and endothelial cell dysfunction by targeting mitophagy in human thoracic aortic dissection patient induced pluripotent stem cell (iPSC) models.Journal of advanced research · 2025Article
- Rewriting the vascular script: epigenetic modifiers as scribes of metabolic reprogramming in pulmonary hypertension.Journal of molecular medicine (Berlin, Germany) · 2025Review
- Pathophysiology of Pulmonary Arterial Hypertension: Focus on Vascular Endothelium as a Potential Therapeutic Target.International journal of molecular sciences · 2025Review
- Article
- Role of BMPR2 Mutation in Lung Organoid Differentiation.Biomedicines · 2025Article
- Bioinformatics Analysis Identifies a Potential Key Gene in the Pathogenesis of Pulmonary Hypertension-HSPH1.Pulmonary circulation · 2025Article
- Experimental animal models and patient-derived platforms to bridge preclinical discovery and translational therapeutics in pulmonary arterial hypertension.Journal of translational medicine · 2025Review
- Isoform-Specific Roles of NTRK2 in Pulmonary Vascular Regeneration.bioRxiv : the preprint server for biology · 2025Article
- Altered maturation and activation state of circulating monocytes is associated with their enhanced recruitment in pulmonary arterial hypertension.Respiratory research · 2025Article
- M2 macrophages-derived exosomes for osteonecrosis of femoral head treatment: modulating neutrophil extracellular traps formation and endothelial phenotype transition.Bone research · 2025Article
- Engineering aortic valves via transdifferentiating fibroblasts into valvular endothelial cells without using viruses or iPS cells.Bioactive materials · 2025Article
- Emerging biomaterials and bio-nano interfaces in pulmonary hypertension therapy: transformative strategies for personalized treatment.Frontiers in bioengineering and biotechnology · 2025Review
87 more citing papers are in PubMed but not listed here.
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Authors and funding
21 authors at 3 institutions in 1 country.
Mingxia GuVera Moulton Wall Center for Pulmonary Vascular Diseases, Stanford University School of Medicine, Stanford, CA 94305, USA; Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Pediatrics, Stanford University School of Medicine, Stanford, CA 94305, USA.
Ning-Yi ShaoStanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA.
Silin SaVera Moulton Wall Center for Pulmonary Vascular Diseases, Stanford University School of Medicine, Stanford, CA 94305, USA; Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Pediatrics, Stanford University School of Medicine, Stanford, CA 94305, USA.
Dan LiVera Moulton Wall Center for Pulmonary Vascular Diseases, Stanford University School of Medicine, Stanford, CA 94305, USA; Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Pediatrics, Stanford University School of Medicine, Stanford, CA 94305, USA.
Vittavat TermglinchanStanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA.
Mohamed AmeenStanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA.
Ioannis KarakikesStanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA.
Gustavo SosaVera Moulton Wall Center for Pulmonary Vascular Diseases, Stanford University School of Medicine, Stanford, CA 94305, USA; Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Pediatrics, Stanford University School of Medicine, Stanford, CA 94305, USA.
Fabian GrubertDepartment of Genetics, Stanford University School of Medicine, Stanford, CA 94305, USA.
Jaecheol LeeStanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA.
Aiqin CaoVera Moulton Wall Center for Pulmonary Vascular Diseases, Stanford University School of Medicine, Stanford, CA 94305, USA; Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Pediatrics, Stanford University School of Medicine, Stanford, CA 94305, USA.
Shalina TaylorVera Moulton Wall Center for Pulmonary Vascular Diseases, Stanford University School of Medicine, Stanford, CA 94305, USA; Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Pediatrics, Stanford University School of Medicine, Stanford, CA 94305, USA.
Yu MaStanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA.
Zhixin ZhaoDepartment of Genetics, Stanford University School of Medicine, Stanford, CA 94305, USA.
James ChappellDepartment of Genetics, Stanford University School of Medicine, Stanford, CA 94305, USA.
Rizwan HamidDepartment of Pediatrics, Vanderbilt University, Nashville, TN 37232, USA.
Eric D AustinDepartment of Pediatrics, Vanderbilt University, Nashville, TN 37232, USA.
Joseph D GoldStanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA.
Joseph C WuStanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA. Electronic address: joewu@stanford.edu.
Michael P SnyderStanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Genetics, Stanford University School of Medicine, Stanford, CA 94305, USA. Electronic address: mpsnyder@stanford.edu.
Marlene RabinovitchVera Moulton Wall Center for Pulmonary Vascular Diseases, Stanford University School of Medicine, Stanford, CA 94305, USA; Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA 94305, USA; Department of Pediatrics, Stanford University School of Medicine, Stanford, CA 94305, USA. Electronic address: marlener@stanford.edu.
Stanford University · USCardiovascular Institute of the South · USVanderbilt University · US
Funding
Vanderbilt Center for Undiagnosed Diseases (VCUD) - BiorepositoryU01HG007674 · NHGRI · VANDERBILT UNIVERSITY MEDICAL CENTER · PI COGAN, JOY D, HAMID, RIZWAN · 2014 to 2022
$13.7MiPSC Derived EC as Surrogates Using Pulmonary Hypertension as a Prototype DiseaseU01HL107393 · NHLBI · STANFORD UNIVERSITY · PI RABINOVITCH, MARLENE, SNYDER, MICHAEL P. · 2011 to 2015
$8.7MIntegrative Omics as a Discovery Tool for Pulmonary HypertensionR01HL122887 · NHLBI · STANFORD UNIVERSITY · PI NICOLLS, MARK ROBERT, RABINOVITCH, MARLENE · 2015 to 2018
$4.1MRole of BMPR2 expression in HPAH; implications for novel therapeutic approachesR01HL102020 · NHLBI · VANDERBILT UNIVERSITY MEDICAL CENTER · PI HAMID, RIZWAN · 2011 to 2015
$2.3MStanford Career Development Program in Omics of Lung DiseasesK12HL120001 · NHLBI · STANFORD UNIVERSITY · PI NICOLLS, MARK ROBERT, RABINOVITCH, MARLENE · 2013 to 2017
$1.3MUncovering compensatory mechanisms in family members with disease causing mutations of pulmonary hypertensionK99HL135258 · NHLBI · STANFORD UNIVERSITY · PI GU, MINGXIA · 2017 to 2018
$336kNHGRI NIH HHS U01 HG007674NHLBI NIH HHS K12 HL120001NHLBI NIH HHS K99 HL135258NHLBI NIH HHS R01 HL102020NHLBI NIH HHS R01 HL122887NHLBI NIH HHS U01 HL107393
Abstract
In familial pulmonary arterial hypertension (FPAH), the autosomal dominant disease-causing BMPR2 mutation is only 20% penetrant, suggesting that genetic variation provides modifiers that alleviate the disease. Here, we used comparison of induced pluripotent stem cell-derived endothelial cells (iPSC-ECs) from three families with unaffected mutation carriers (UMCs), FPAH patients, and gender-matched controls to investigate this variation. Our analysis identified features of UMC iPSC-ECs related to modifiers of BMPR2 signaling or to differentially expressed genes. FPAH-iPSC-ECs showed reduced adhesion, survival, migration, and angiogenesis compared to UMC-iPSC-ECs and control cells. The "rescued" phenotype of UMC cells was related to an increase in specific BMPR2 activators and/or a reduction in inhibitors, and the improved cell adhesion could be attributed to preservation of related signaling. The improved survival was related to increased BIRC3 and was independent of BMPR2. Our findings therefore highlight protective modifiers for FPAH that could help inform development of future treatment strategies.
Indexed as
Base SequenceBone Morphogenetic Protein 4Bone Morphogenetic Protein Receptors, Type IICell AdhesionCell MovementCell ShapeCell SurvivalEndothelial CellsGene EditingGene Expression RegulationHeterozygoteHumansHypertension, PulmonaryInduced Pluripotent Stem CellsMutationNeovascularization, PhysiologicBMPR2 protein, humanBone Morphogenetic Protein 4Bone Morphogenetic Protein Receptors, Type IIp38 Mitogen-Activated Protein KinasesSmad Proteinsbone morphogenetic protein receptor 2cell adhesioncell signalingcell survivalendothelial dysfunctioninduced pluripotent stem cell-derived endothelial cellpenetrancepulmonary arterial hypertensiontranscriptomic analysisunaffected mutation carrier
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