ArticleAmerican journal of respiratory and critical care medicine2016
Bone Marrow-derived Cells Contribute to the Pathogenesis of Pulmonary Arterial Hypertension.
Article in American journal of respiratory and critical care medicine, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 49 papers, 1 of them a synthesis that pooled it.
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Who cites it
49 citing papers in PubMed, 1 synthesis or guideline pooled it, 67 citations in OpenAlex.
- Meta-analysis of blood genome-wide expression profiling studies in pulmonary arterial hypertension.American journal of physiology. Lung cellular and molecular physiology · 2020Pooled it
- Beyond the Heart-Lung Axis: A Review of the Crosstalk Between the Hemodynamic, Neurohormonal, and Immune Systems in Pulmonary Hypertension.Comprehensive physiology · 2026Review
- Flow Cytometric Determination of Circulating Progenitor Cells in Patients With Pulmonary Arterial Hypertension: A Systematic Review.Pulmonary circulation · 2025Review
- Reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertension.ERJ open research · 2025Article
- Pulmonary arterial hypertension in children with congenital heart disease: a deeper look into the role of endothelial progenitor cells and circulating endothelial cells to assess disease severity.Frontiers in pediatrics · 2023Article
- Light at the ENDothelium-role of Sox17 and Runx1 in endothelial dysfunction and pulmonary arterial hypertension.Frontiers in cardiovascular medicine · 2023Review
- Plasma Cell-Free DNA Predicts Survival and Maps Specific Sources of Injury in Pulmonary Arterial Hypertension.Circulation · 2022Article
- New Mutations and Pathogenesis of Pulmonary Hypertension: Progress and Puzzles in Disease Pathogenesis.Circulation research · 2022Review
- Potential biomarkers and therapeutic targets of idiopathic pulmonary arterial hypertension.Physiological reports · 2022Article
- KCNK3 Mutation Causes Altered Immune Function in Pulmonary Arterial Hypertension Patients and Mouse Models.International journal of molecular sciences · 2021Article
- Gamma heavy chain disease complicated by pulmonary hypertension, which was successfully treated with lenalidomide.BMJ case reports · 2020Article
- Long Range Endocrine Delivery of Circulating miR-210 to Endothelium Promotes Pulmonary Hypertension.Circulation research · 2020Article
- The hallmarks of severe pulmonary arterial hypertension: the cancer hypothesis-ten years later.American journal of physiology. Lung cellular and molecular physiology · 2020Review
- Prevalence of pulmonary hypertension in myelofibrosis.Annals of hematology · 2020Article
- Nonclassical Monocytes Sense Hypoxia, Regulate Pulmonary Vascular Remodeling, and Promote Pulmonary Hypertension.Journal of immunology (Baltimore, Md. : 1950) · 2020Article
- Pluripotent hematopoietic stem cells augment α-adrenergic receptor-mediated contraction of pulmonary artery and contribute to the pathogenesis of pulmonary hypertension.American journal of physiology. Lung cellular and molecular physiology · 2020Article
- Long-term Effects of the Janus Kinase 1/2 Inhibitor Ruxolitinib on Pulmonary Hypertension and the Cardiac Function in a Patient with Myelofibrosis.Internal medicine (Tokyo, Japan) · 2020Article
- Low dose 100 cGy irradiation as a potential therapy for pulmonary hypertension.Journal of cellular physiology · 2019Article
- Machine learning-based microarray analyses indicate low-expression genes might collectively influence PAH disease.PLoS computational biology · 2019Article
- The Search for Disease-Modifying Therapies in Pulmonary Hypertension.Journal of cardiovascular pharmacology and therapeutics · 2019Review
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Authors and funding
12 authors at 4 institutions in 1 country.
Funding
Abstract
rationalePulmonary arterial hypertension (PAH) is a progressive lung disease of the pulmonary microvasculature. Studies suggest that bone marrow (BM)-derived circulating cells may play an important role in its pathogenesis.
objectivesWe used a genetic model of PAH, the Bmpr2 mutant mouse, to study the role of BM-derived circulating cells in its pathogenesis.
methodsRecipient mice, either Bmpr2(R899X) mutant or controls, were lethally irradiated and transplanted with either control or Bmpr2(R899X) BM cells. Donor cells were traced in female recipient mice by Y chromosome painting. Molecular and function insights were provided by expression and cytokine arrays combined with flow cytometry, colony-forming assays, and competitive transplant assays. MEASUREMENTS AND MAIN
resultsWe found that mutant BM cells caused PAH with remodeling and inflammation when transplanted into control mice, whereas control BM cells had a protective effect against the development of disease, when transplanted into mutant mice. Donor BM-derived cells were present in the lungs of recipient mice. Functional and molecular analysis identified mutant BM cell dysfunction suggestive of a PAH phenotype soon after activation of the transgene and long before the development of lung pathology.
conclusionsOur data show that BM cells played a key role in PAH pathogenesis and that the transplanted BM cells were able to drive the lung phenotype in a myeloablative transplant model. Furthermore, the specific cell types involved were derived from hematopoietic stem cells and exhibit dysfunction long before the development of lung pathology.
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