Pulmonary Hypertension Research and Treatments

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

This study uses patient-derived iSMCs to uncover new mechanisms of PAH and sotatercept's effects beyond its known anti-proliferative actions.

Genetic hallmarks of patients with congenital portosystemic shunts and portopulmonary hypertension: protocol for a multicentre exploratory study

This paper outlines a protocol to identify genetic variants associated with portopulmonary hypertension in congenital portosystemic shunt patients using whole-genome sequencing and family-based analyses.

A GCN1-independent activator of the kinase GCN2

The paper identifies three GCN2 activators, one of which works independently of GCN1, and describes its unique binding mechanism.

A Single-Cell Atlas of PVOD Across the Lifespan Reveals Cellular and Molecular Therapeutic Targets

The paper constructs a single-cell atlas of PVOD across the lifespan, identifying endothelial dysfunction and molecular pathways as key drivers of the disease with potential therapeutic targets.

VPS34 regulation in pulmonary arterial vascular smooth muscle cells in pulmonary hypertension

This study investigates VPS34 activation in pulmonary arterial vascular smooth muscle cells in pulmonary hypertension, finding it drives proliferation and fibrotic remodeling, and shows SAR405 inhibition reduces disease progression.

DLGAP5 promotes vascular cell proliferation and remodeling in pulmonary arterial hypertension

This study identifies DLGAP5 as a driver of pulmonary vascular remodeling in PAH by promoting cell proliferation and survival through cyclin A2 and DEPDC1 upregulation and BIM suppression.

TREM2 Orchestrates Myeloid Cell Programming and Immune Dysregulation in Pulmonary Hypertension

This study identifies TREM2 as a regulator of myeloid cell function and immune dysregulation in pulmonary hypertension, showing its role in disease progression and potential as a therapeutic target.

Multi-Omic Profiling Defines the Renal Mechanisms of Cardiovascular-Kidney-Metabolic Syndrome in Pulmonary Arterial Hypertension

This study uses multi-omic profiling to identify renal mechanisms in PAH, revealing structural and metabolic changes and potential therapeutic targets.

Sexually dimorphic role of estrogen receptor α in preserving right ventricular endothelial integrity

This study shows estrogen receptor α protects female RV endothelial cells from apoptosis and supports angiogenesis in pulmonary hypertension, revealing a sex-specific role in RV function.

17β-Estradiol Promotes Right Ventricle Angiogenesis via Estrogen Receptor α and Apelin Signaling

This study shows that 17β-estradiol promotes right ventricle angiogenesis through estrogen receptor α and apelin signaling in pulmonary hypertension.

Norepinephrine Induces Proliferation and Migration of Human Pulmonary Artery Smooth Muscle Cells via Endothelin 1

This study shows that norepinephrine promotes proliferation and migration of human pulmonary artery smooth muscle cells through the endothelin-1 signaling pathway and JNK/c-jun pathway.

Predictive vascular growth and remodeling in pulmonary hypertension: simulating intervention effects from captured evolution

This paper develops a multiscale computational model of pulmonary vascular remodeling in PH, showing it can predict pressure, wall thickness, and stiffness changes in response to treatment.

Liriodendrin Targets PFKFB3 to Suppress Inflammatory Phenotypic Transition and Vascular Remodeling in Pulmonary Hypertension

This study identifies liriodendrin as a compound that targets PFKFB3 to reduce inflammation and vascular remodeling in pulmonary hypertension.

CD133+ progenitor cells promote pulmonary hypertension through CXCR4 signaling

This study identifies CD133+ progenitor cells as contributors to pulmonary hypertension and shows that CXCR4 signaling drives their pathogenic role in vascular remodeling.

Multicellular Programs Associated with Right Ventricular Adaptation in Pulmonary Arterial Hypertension

The paper identifies multicellular molecular programs associated with RV adaptation in PAH using single-nucleus RNA sequencing and integrates them with physiological measurements.

Paracrine Action of Bone Morphogenetic Protein 3 in Pulmonary Arterial Hypertension

This study shows that BMP3 overexpression reduces pulmonary vascular remodeling and cardiac dysfunction in PAH by balancing TGF-β and BMP pathways through paracrine signaling.

Endothelial β3-Adrenergic Receptor activation prevents pulmonary hypertension

This study shows that activating the endothelial β3-adrenergic receptor prevents pulmonary hypertension by improving vascular function and reducing remodeling.

Pulmonary primary oxysterol and bile acid synthesis as a predictor of outcomesin pulmonary arterial hypertension

The paper identifies pulmonary primary oxysterol and bile acid synthesis as a prognostic biomarker for pulmonary arterial hypertension, outperforming current clinical risk scores.

A Large Animal Model of Heritable Pulmonary Arterial Hypertension UsingGene-edited BMPR2 Sheep

This paper develops a gene-edited ovine model of heritable pulmonary arterial hypertension to study BMPR2 mutations and their pathophysiology.

E2F1 Drives Endothelial Arterial Programming in Pulmonary Arterial Hypertension

This study identifies E2F1 as a driver of pulmonary arterial hypertension by linking endothelial cell-cycle activation to arterial programming and vascular remodeling, showing that its inhibition mitigates disease in models.

Deletion of GPR39 Prevents Pulmonary Arterial Hypertension by Attenuating Hypoxia-Induced Aberrant Signaling

This study shows that deleting GPR39 prevents pulmonary arterial hypertension by reducing hypoxia-induced signaling and vascular remodeling.

Endothelial Baf60c in BPD-Associated Pulmonary Hypertension

This study investigates the role of Baf60c in BPD-associated pulmonary hypertension, finding that hyperoxia reduces Baf60c and increases Smarcc2, leading to suppressed PI3K-Akt-mTOR signaling in pulmonary microvascular endothelial cells.

Proteomics Defines Shared and Divergent Alterations in the Right Atrium and Right Ventricle in Porcine Right Heart Failure

The paper uses proteomics to compare alterations in the right atrium and right ventricle of pigs with right heart failure, identifying shared and distinct metabolic changes.

Metabolomic Network Analysis Reveals Reorganization of Lipid and Steroid Programs Linked to Right Ventricular-Pulmonary Vascular Function in Pulmonary Hypertension

The paper identifies metabolic modules in PAH patients linked to RV-PV function using WGCNA and validates findings in independent cohorts.

Targeting pathogenic VWF/ADAMTS13 dysregulation attenuates CTEPH progression

This study identifies VWF/ADAMTS13 dysregulation as a driver of CTEPH progression and shows that targeting this axis reduces thrombosis and vascular remodeling in rat models.

KLF6 in Pulmonary Hypertension: The Dual Role of Friend and Foe

This study identifies KLF6 as a key driver of endothelial dysfunction in pulmonary arterial hypertension, showing its role in promoting vascular remodelling and angioproliferative lesions.

Biological Aging of the Cardiopulmonary System

This study quantifies biological cardiopulmonary aging in mice by integrating biomechanics and single-cell transcriptomics, revealing non-linear aging patterns and cell-type-specific gene expression changes.

Sex-linked Lung Estrobolome May Contribute to Pulmonary Hypertension Penetrance of Bmpr2 R899X Mutation via an ET-1high Endoregulatory Macrophage Phenotype

This study identifies a microbiome-estrogen-immune axis that contributes to sex-linked pulmonary hypertension in Bmpr2 R899X mutation carriers.

Baseline State for Pulmonary Vasculature with Pulmonary Arterial Hypertension: Effect of Geometric Remodeling and Metabolic Shift

This study develops a computational framework to establish a baseline state of pulmonary vasculature in PAH, comparing it with healthy vasculature in terms of mechanics, morphology, and hemodynamics.

Impaired Lung BCAA Metabolism Promotes Ferroptosis and Resultant Pulmonary Arterial Hypertension-Associated Hepatopathy

This study shows that impaired BCAA metabolism promotes ferroptosis, leading to pulmonary arterial hypertension and associated liver disease, and that targeting BCAA metabolism can alleviate these effects.