Pulmonary Hypertension Research and Treatments

Pulmonary Arterial Hypertension Induces a Metabolic and Inflammatory Hepatopathy

This study identifies metabolic and inflammatory changes in the liver of PAH patients using single nucleus RNA sequencing, revealing distinct cellular phenotypes and signaling pathways.

Identification of drug candidates for rescue of SOX17 gene targets in pulmonary arterial hypertension

The paper identifies BX-912 as a compound that rescues SOX17 gene targets and prevents pulmonary hypertension in mice.

Impaired SOX17 Expression Causes Endothelial Dysfunction and Pulmonary Arterial Hypertension by Insufficient Suppression of RUNX1

This study shows that impaired SOX17 expression increases pulmonary arterial hypertension risk by failing to suppress RUNX1, leading to endothelial dysfunction, and that RUNX1 inhibition can mitigate this condition.

Pulmonary Fibrosis Enhances Vasodilation to Calcitonin Gene-Related Peptide

This study shows that pulmonary fibrosis enhances CGRP-induced hyperpolarization and vasodilation in male mice through PKA-dependent KATP channel activation, with no effect in females.

Imatinib Reduces Right Ventricular Systolic Pressure Independent of Arterial or Venous Remodeling in an Inflammatory Murine Model of Pulmonary Hypertension

This study shows imatinib lowers right ventricular systolic pressure without affecting vascular remodeling in a mouse model of pulmonary hypertension.

Targeting IL-6-STAT3/STAT4 Signaling Restores FOXP3 Expression in Pulmonary Arterial Endothelium and Reveals Novel Biomarkers for PAH

This study shows that blocking IL-6 restores FOXP3 expression in PAH, revealing its role as a biomarker and therapeutic target.

A chimeric human-mouse lung vascular model using induced pluripotent stem cells reveals insights into the pathogenesis of BMPR2-related pulmonary hypertension

The paper develops a chimeric human-mouse lung vascular model using hiPSCs to study BMPR2-related pulmonary hypertension, revealing novel molecular and functional defects in human endothelial cells.

Pulmonary Hypertension Engine for Linked Experiments (PHELEX): a platform for the re-analysis of public transcriptomic data related to pulmonary hypertension in both animal models, and humans.

PHELEX provides a searchable repository of RNA sequencing data for pulmonary hypertension, enabling analysis and cross-study comparisons to uncover molecular signatures.

CD248 activates TGF-β receptor I to promote vascular remodeling in pulmonary arterial hypertension

This study identifies CD248 as a co-activator of TGF-β receptor I in pulmonary arterial hypertension, supporting anti-CD248 therapy as a targeted alternative to systemic TGF-β inhibition.

Excessive Postnatal Smooth Muscle Differentiation in a Lung Specific Model of TBX4-related Pulmonary Hypertension

This study develops a mouse model of TBX4-related pulmonary hypertension and shows excessive postnatal smooth muscle differentiation in multiple lung compartments.

Efficacy of SGLT2 Inhibitors in Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis of Preclinical Studies

This study evaluates the effects of SGLT2 inhibitors in preclinical PAH models, finding significant improvements in pulmonary hemodynamics and right ventricular function.

Chronic therapy with α1A-adrenergic agonist reverses RV failure and mitochondrial dysfunction

This study shows chronic treatment with a 1A-adrenergic agonist improves mitochondrial function and reverses right ventricular failure in a mouse model.

Multimodality Molecular Profiling Nominates Targetable Mechanisms in Progressive RV Dysfunction

This study uses multi-omics profiling to identify molecular mechanisms contributing to progressive right ventricular dysfunction in pigs.

A critical contribution of cardiac myofibroblasts in right ventricular failure and the role of UCP2 SNPs in the predisposition to RV decompensation in pulmonary arterial hypertension

The paper investigates the role of cardiac myofibroblasts and UCP2 SNPs in right ventricular failure in pulmonary arterial hypertension, finding increased cMFB and reduced UCP2 associated with decompensation.

PAH-former: Transfer Learning for Efficient Discovery of Pulmonary Arterial Hypertension-Associated Genes

PAH-former uses transfer learning with Geneformer to identify PAH-associated genes by fine-tuning on public PAH lung tissue data and performing in silico perturbation analysis.

Mitochondrial acid-sensing ion channel 1a deficiency induces mitochondrial dysfunction in pulmonary arterial smooth muscle cells

This study shows that loss of mtASIC1a in PASMCs under chronic hypoxia leads to mitochondrial dysfunction and apoptosis resistance.

CES1 DEFICIENCY IS ASSOCIATED WITH METABOLIC REPROGRAMING AND ENDOTHELIAL DYSFUNCTION IN PULMONARY ARTERIAL HYPERTENSION

This study identifies CES1 deficiency as a driver of metabolic reprogramming and endothelial dysfunction in pulmonary arterial hypertension, showing its role in lipid metabolism and oxidative stress.

Cardiomyocyte NLRP3 signaling in right heart failure is sexually dimorphic via estrogen receptor α

This study identifies a sexually dimorphic NLRP3 signaling pathway in right ventricular failure, showing estrogen receptor α mediates protection against NLRP3-induced contractile dysfunction in cardiomyocytes.

LOSS OF ROR2 TYROSINE KINASE RECEPTOR IS ASSOCIATED WITH ENDOTHELIAL DYSFUNCTION IN PAH VIA INAPPROPRIATE INTEGRIN BETA 1 ACTIVATION

This study shows that loss of ROR2 leads to endothelial dysfunction in PAH through inappropriate integrin beta 1 activation, impairing barrier integrity and angiogenesis.

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

This study shows that Sotatercept reverses SIN3a deficiency-driven pulmonary arterial hypertension by reprogramming BMPR2/TGF-β-HIF-1α signaling pathways.

Nicotinamide phosphoribosyltransferase activates the mitochondrial unfolded protein response to promote pulmonary arterial endothelial cell proliferation

This study shows that Nampt activates the mitochondrial unfolded protein response to enhance pulmonary arterial endothelial cell proliferation in PAH.

Interrogation of the glycolytic axis in pulmonary arterial hypertension models and how modifying these can lead to potential therapeutic options.

This paper investigates the glycolytic axis in PAH models and shows that TEPP-46 treatment reduces nuclear PKM2 and improves PAH symptoms in rodent models.

Heterogeneous Nuclear Ribonucleoprotein A1 as a Key Regulator in Pulmonary Arterial Hypertension Development

This study identifies differentially expressed proteins in pulmonary arterial hypertension lesions, highlighting hnRNPA1 as a key regulator in plexiform lesion formation.

Sex difference in rat ventilatory response and pulmonary vascular resistance

This study examines sex differences in rat ventilation and pulmonary vascular resistance, finding no sex difference in ventilatory response but higher pulmonary vascular resistance in males.

Monocrotaline treatment of the rat predisposes to altered lipopolysaccharide-induced lung responses.

This study shows that monocrotaline-treated rats exhibit exaggerated lung responses to lipopolysaccharide, characterized by pro-inflammatory gene expression and increased thrombotic factors.

Endothelial GATA6 deficiency suppresses intracellular TLR3-interferon signaling in HPAECs and promotes interferon response in HPASMCs

This study shows that GATA6 deficiency in endothelial cells reduces TLR3-interferon signaling and promotes interferon responses in adjacent smooth muscle cells through paracrine effects.

TXNDC5 Governs Extracellular Matrix Homeostasis in Pulmonary Hypertension

This study identifies TXNDC5 as a key regulator of extracellular matrix homeostasis in pulmonary hypertension, showing its role in vascular remodeling and potential as a therapeutic target.

Lactate dehydrogenase-induced DNA Topoisomerase 1 is a novel regulator of smooth muscle cell proliferation and remodeling in pulmonary arterial hypertension

This study identifies LDHA-lactate-induced over-lactylation and TOP1 accumulation as drivers of PASMC hyper-proliferation and PAH, with TOP1 inhibition reversing these effects.

Na+/H+ Exchanger Isoform 1 Regulates Apoptosis Susceptibility in Pulmonary Arterial Smooth Muscle from the Sugen/Hypoxia model of Pulmonary Hypertension

This study shows NHE1 promotes apoptosis resistance in PASMCs from PH models through p38 suppression, independent of ion transport or ezrin-binding.

Endothelial-targeted CD39 prevents Toxin-induced Pulmonary Hypertension Mice.

This paper shows that targeting CD39 on endothelial cells prevents toxin-induced pulmonary hypertension in mice by multiple mechanisms including adenosine signaling and reducing inflammation.