Pulmonary Hypertension Research and Treatments

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

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.

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

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

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

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.

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.

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.

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.

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.

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

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

This study identifies CTRP7 as a biomarker for predicting responsiveness to pulmonary vasodilators in PAH by linking its expression to IL-6 and PTGIR regulation in human and mouse models.

This study investigates adaptive right ventricular development in congenital heart disease-associated pulmonary arterial hypertension, finding preserved microvascular density and increased cardiomyocyte hyperplasia support RV function.

This study shows that PKR-mediated integrated stress response drives PVOD pathogenesis in mice, with PKR knockout preventing vascular remodeling and pulmonary hypertension.

This study identifies LINC00599 as a key regulator of pulmonary hypertension through m6A modification and liquid-liquid phase separation with G3BP1 and MYH9, promoting PASMC proliferation.

This study uses a 1D-FSI model to quantify how vascular stiffening and resistance contribute to increased MPA pressure in pulmonary hypertension.

This study identifies lncRNA-536 and RBM25 interactions that regulate HOXB13 expression, contributing to PASMC hyperproliferation in pulmonary arterial hypertension.

The paper identifies FABP4/5 as key regulators of endothelial glycolysis and arterial programming in pulmonary hypertension using single-cell and spatial transcriptomics.

This study shows Lactobacillus modulates the gut microbiome to reduce inflammation and improve RV function in PAH by counteracting GP130-mediated microtubule remodeling.

This paper develops a 3D-bioprinted model of pulmonary artery adventitia using female patient-derived cells to show that estrogen signaling mediates fibroblast activation in PAH, with implications for drug testing.

This study examines structural and mechanical changes in the main pulmonary artery in pulmonary arterial hypertension and links them to impaired right ventricular function and coupling.

This study investigates endothelial features in CTEPH, finding that while VEGF-A/VEGFR2 and COL15A1 expression differ between lesion types, in vitro angiogenic and barrier functions of PAECs are similar.

This study shows that loss of Tbx4 in embryonic lung mesenchyme leads to postnatal lung developmental defects and increased pulmonary hypertension.

This study shows that inhibiting NHE-1 with rimeporide reduces right ventricular hypertrophy, pulmonary vascular remodelling, inflammation, and fibrosis in a rat model of pulmonary arterial hypertension.