ArticleAmerican journal of respiratory and critical care medicine2019
Bone Morphogenetic Protein 9 Is a Mechanistic Biomarker of Portopulmonary Hypertension.
Article in American journal of respiratory and critical care medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 59 papers, 1 of them a synthesis that pooled it.
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Who cites it
59 citing papers in PubMed, 1 synthesis or guideline pooled it, 107 citations in OpenAlex.
- International Liver Transplantation Society practice guideline update on portopulmonary hypertension.Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society · 2026Guideline
- Activin Signaling Inhibitors in Pulmonary Hypertension: A State-of-the-Art Review.Pulmonary circulation · 2026Review
- The Importance of Liver-Lung Communication in Pulmonary Vascular Diseases.Comprehensive Physiology · 2026Review
- Article
- Transpulmonary Proteome Gradients Identify Pathways Involved in Pulmonary Vascular Disease Due To Heart Failure.Circulation. Heart failure · 2025Observational
- Disrupting BMP/TGF-β Signaling: Modulation of AQP1 and TGFB1 in Human Pulmonary Microvascular Endothelial Cells.Comprehensive Physiology · 2025Article
- Bmpr2 Loss in the Vascular Endothelium Enhances Neoangiogenesis and Growth of Lung Metastatic Lesions in a Mouse Model of Breast Cancer.Journal of cellular biochemistry · 2025Article
- Clinical aspects encountered in patients with portopulmonary hypertension.Medicine and pharmacy reports · 2025Review
- BMP9 regulates the endothelial secretome to drive pulmonary hypertension.bioRxiv : the preprint server for biology · 2025Article
- Transpulmonary bubble transit in patients hospitalised with COVID-19 pneumonia.BMJ open respiratory research · 2025Article
- Can Plasma ADAMTS13 Differentiate Patients With Pulmonary Arterial Hypertension From Other Forms of Pulmonary Hypertension and Dyspnea Control Patients.CHEST pulmonary · 2025Article
- Bone morphogenetic protein-9 controls pulmonary vascular growth and remodeling.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Therapeutic targets for pulmonary arterial hypertension: insights into the emerging landscape.Expert opinion on therapeutic targets · 2025Review
- Observational
- Advances in intrahepatic and extrahepatic vascular dysregulations in cirrhotic portal hypertension.Frontiers in medicine · 2025Review
- Circulating biomarkers in pulmonary arterial hypertension: State-of-the-art review and future directions.JHLT open · 2024Review
- Pathology and pathobiology of pulmonary hypertension: current insights and future directions.The European respiratory journal · 2024Review
- Hepatopulmonary Syndrome, Another Face of Dysregulated BMP9 Signaling.American journal of respiratory and critical care medicine · 2024Article
- Unveiling the Impact of BMP9 in Liver Diseases: Insights into Pathogenesis and Therapeutic Potential.Biomolecules · 2024Review
- Effects of Modulating BMP9, BMPR2, and AQP1 on BMP Signaling in Human Pulmonary Microvascular Endothelial Cells.International journal of molecular sciences · 2024Article
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Authors and funding
27 authors at 10 institutions in 3 countries.
Funding
Abstract
rationaleBMP9 (bone morphogenetic protein 9) is a circulating endothelial quiescence factor with protective effects in pulmonary arterial hypertension (PAH). Loss-of-function mutations in BMP9, its receptors, and downstream effectors have been reported in heritable PAH.
objectivesTo determine how an acquired deficiency of BMP9 signaling might contribute to PAH.
methodsPlasma levels of BMP9 and antagonist soluble endoglin were measured in group 1 PAH, group 2 and 3 pulmonary hypertension (PH), and in patients with severe liver disease without PAH. MEASUREMENTS AND MAIN
resultsBMP9 levels were markedly lower in portopulmonary hypertension (PoPH) versus healthy control subjects, or other etiologies of PAH or PH; distinguished PoPH from patients with liver disease without PAH; and were an independent predictor of transplant-free survival. BMP9 levels were decreased in mice with PH associated with CCl
conclusionsBMP9 is a sensitive and specific biomarker of PoPH, predicting transplant-free survival and the presence of PAH in liver disease. In rodent models, acquired deficiency of BMP9 signaling can predispose to or exacerbate PH, providing a possible mechanistic link between PoPH and heritable PAH. These findings describe a novel experimental model of severe PH that provides insight into the synergy between pulmonary vascular injury and diminished BMP9 signaling in the pathogenesis of PAH.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.