ReviewBiochemical Society transactions2024
Bone morphogenetic protein signalling in pulmonary arterial hypertension: revisiting the BMPRII connection.
Review in Biochemical Society transactions, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed.
- Sodium Selenite Attenuates Monocrotaline-Induced Pulmonary Arterial Hypertension by Upregulating GPX3 and Ameliorating Vascular Remodeling.Physiological research · 2026Article
- Emerging significance of E3 ubiquitin ligases and Deubiquitinases in pulmonary hypertension.Molecular medicine (Cambridge, Mass.) · 2026Review
- Sex-Specific Pathophysiological Signatures in Allometric Dosing-Controlled Bleomycin Acute Lung Injury Model.bioRxiv : the preprint server for biology · 2026Article
- Article
- BMP7 attenuates myocardial injury and preserves cardiac function after myocardial infarction by inhibiting cardiomyocyte pyroptosis.Apoptosis : an international journal on programmed cell death · 2026Article
- Sotatercept in Pulmonary Arterial Hypertension: Molecular Mechanisms, Clinical Evidence, and Emerging Role in Reverse Remodelling.International journal of molecular sciences · 2026Review
- Deciphering the Counterintuitive Role of Vascular Endothelial Growth Factor Signaling Pathways in Pulmonary Arterial Hypertension.International journal of molecular sciences · 2026Review
- Case Report: Pulmonary artery biopsy findings in a patient with a BMPR2 variant-associated pulmonary arterial hypertension.Frontiers in medicine · 2026Article
- Transcriptomic profiling of the sex-linked biological pathways of severe pulmonary arterial hypertension associated with endothelial cell caveolin-1 depletion and chronic hypoxia.Frontiers in physiology · 2026Article
- Disrupting BMP/TGF-β Signaling: Modulation of AQP1 and TGFB1 in Human Pulmonary Microvascular Endothelial Cells.Comprehensive Physiology · 2025Article
- Sotatercept: A Crosstalk Between Pathways and Activities in the Pulmonary Circulation and Blood.International journal of molecular sciences · 2025Review
- Distinct signalling dynamics of BMP4 and BMP9 in brown versus white adipocytes.Scientific reports · 2025Article
- Review
- Article
- Exosomal BMPR2 Macromolecule Facilitates Alveolar Epithelial Cell Repair Through Functional Complex Formation with BMPR1B in Acute Lung Injury.International journal of nanomedicine · 2025Article
- In vitro and in vivo characterization of wild type BMP9 and a non-osteogenic variant in models of pulmonary arterial hypertension.PloS one · 2025Article
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Authors and funding
2 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pulmonary arterial hypertension (PAH) is a rare and life-threatening vascular disorder, characterised by abnormal remodelling of the pulmonary vessels and elevated pulmonary artery pressure, leading to right ventricular hypertrophy and right-sided heart failure. The importance of bone morphogenetic protein (BMP) signalling in the pathogenesis of PAH is demonstrated by human genetic studies. Many PAH risk genes are involved in the BMP signalling pathway and are highly expressed or preferentially act on vascular endothelial cells. Endothelial dysfunction is recognised as an initial trigger for PAH, and endothelial BMP signalling plays a crucial role in the maintenance of endothelial integrity. BMPR2 is the most prevalent PAH gene, found in over 80% of heritable cases. As BMPRII protein is the major type II receptor for a large family of BMP ligands and expressed ubiquitously in many tissues, dysregulated BMP signalling in other cells may also contribute to PAH pathobiology. Sotatercept, which contains the extracellular domain of another transforming growth factor-β family type II receptor ActRIIA fused to immunoglobin Fc domain, was recently approved by the FDA as a treatment for PAH. Neither its target cells nor its mechanism of action is fully understood. This review will revisit BMPRII function and its extracellular regulation, summarise how dysregulated BMP signalling in endothelial cells and smooth muscle cells may contribute to PAH pathogenesis, and discuss how novel therapeutics targeting the extracellular regulation of BMP signalling, such as BMP9 and Sotatercept, can be related to restoring BMPRII function.
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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.