A multicentre study recently published on the European Respiratory Journal Open Research looked at bone morphogenetic protein receptor 2 (BMPR2) signalling — a pathway already known to be disrupted in heritable pulmonary arterial hypertension (PAH) — across a wider range of pulmonary hypertension (PH) patients, to see whether the same disturbance shows up in other pulmonary arterial hypertension subtypes and in chronic thromboembolic pulmonary hypertension (CTEPH). Researchers analysed blood samples from 234 people across seven groups: heritable pulmonary arterial hypertension, idiopathic pulmonary arterial hypertension (with and without comorbidities), systemic sclerosis-associated pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, healthy carriers of bone morphogenetic protein receptor 2 gene variants, and healthy controls.
The findings suggest bone morphogenetic protein receptor 2 signalling impairment may be a broader feature of pulmonary hypertension than previously thought, extending beyond heritable pulmonary arterial hypertension to other pulmonary arterial hypertension subtypes andchronic thromboembolic pulmonary hypertension. The authors propose that BMPR2 mRNA and BMP10 protein levels in blood could serve as new biomarkers for disease severity.
Download the pdf with full text at this link on the European Respiratory Journal Open Research
Citation
Impaired BMPR2 signalling across pulmonary hypertension subgroups: a multicentre study
Simon O. Haas, Nicola Benjamin, Memoona Shaukat, Marc A. Schneider, Sarah Richtmann, Natascha Sommer, Karen M. Olsson, Marius M. Hoeper, Vivienne Theobald, Satenik Harutyunova, Panagiota Xanthouli, Benjamin Egenlauf, Ekkehard Grünig, Christina A. Eichstaedt ERJ Open Research 2026 01644-2025; DOI: https://doi.org/10.1183/23120541.01644-2025