Article

iPSC modeling of pulmonary arterial hypertension to uncover pathomechanisms and unrecognized modes of action of sotatercept

2026-03-13

Abstract excerpt

Pulmonary arterial hypertension (PAH) is a potentially fatal disease characterized by obliterative remodeling of distal pulmonary arteries, commonly associated with bone morphogenetic receptor type 2 (BMPR2) gene mutations. In patients with PAH, sotatercept, an activin signaling inhibitor, improves hemodynamics and outcomes, but clinical responses vary and sometimes occur within weeks, suggesting additional mechan...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: e7a2f4e5-4893-58e8-878c-dec5674e3a09
DOI: 10.64898/2026.03.12.711267

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

iPSC modeling of pulmonary arterial hypertension to uncover pathomechanisms and unrecognized modes of action of sotaterceptDOI 10.64898/2026.03.12.711267Read

Sotatercept Reverses SIN3a Deficiency-Driven PAH by Reprogramming BMPR2/TGF-β-HIF-1α Signaling PathwaysSemantic proximity Read

Emerging Mechanistic Insights and Therapeutic Strategies for Pulmonary Arterial Hypertension: A Focus on Right Ventricular Dysfunction and Novel Treatment PathwaysSemantic proximity Read

Developing an induced pluripotent stem cell model of pulmonary arterial hypertension to understand the contribution of BMPR2 mutations to disease-associated phenotypes in smooth muscle cellsSemantic proximity Read

Patient-Specific iPSC-Derived Endothelial Cells Uncover Pathways that Protect against Pulmonary Hypertension in BMPR2 Mutation Carriers.Semantic proximity Read

Genomic, transcriptomic and proteomic depiction of iPSC-derived smooth muscle cells as emerging cellular models for arterial diseasesSemantic proximity Read

Consistent Safety and Efficacy of Sotatercept for Pulmonary Arterial Hypertension in BMPR2 Mutation Carriers and Noncarriers: A Planned Analysis of a Phase II, Double-Blind, Placebo-controlled Clinical Trial (PULSAR).Semantic proximity Read

Vascular Smooth Muscle Cells From Hypertensive Patient-Derived Induced Pluripotent Stem Cells to Advance Hypertension PharmacogenomicsSemantic proximity Read

iPSC–endothelial cell phenotypic drug screening and in silico analyses identify tyrphostin-AG1296 for pulmonary arterial hypertensionSemantic proximity Read

Developments in therapeutics for pulmonary arterial hypertension.Semantic proximity Read

Management of Pulmonary Arterial Hypertension. Current Strategies and Future ProspectsSemantic proximity Read

BMP9 regulates the endothelial secretome to drive pulmonary hypertensionSemantic proximity Read

An emerging class of new therapeutics targeting TGF , Activin, and BMP ligands in pulmonary arterial hypertensionSemantic proximity Read