Pulmonary Hypertension Research and Treatments

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.

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

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.

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.

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

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

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.

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

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.

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

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

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

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

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 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.

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

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.

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.

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

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

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

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.

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

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.

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

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.

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.

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.

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

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.