Pulmonary Hypertension Research and Treatments

This study identifies myeloid cell-derived IL1β as a key driver of pulmonary vascular remodeling in HFpEF using a murine model and multi-omics approaches.

This paper classifies PAH-related genes based on evidence strength using a semi-quantitative scoring system, identifying twelve genes with definitive evidence and recommending their inclusion in genetic testing panels.

The paper identifies a Tm4sf1-marked endothelial stem/progenitor cell subpopulation in rat lungs associated with PAH and shows its relevance to human PAH through transcriptomic analysis.

This study evaluates a beta-arrestin-biased AT1R agonist in PAH models, finding it fails to improve survival compared to AngII but promotes vascular remodeling.

This study shows preventive exercise in rats with pulmonary arterial hypertension increases collagen I and myosin 7 mRNA expression, suggesting it modulates extracellular matrix and myosin gene expression.

This study identifies two new PAH risk genes, FBLN2 and PDGFD, through rare variant analysis of 4,241 PAH cases, with de novo variants contributing to ~15% of pediatric IPAH.

This study shows that loss of FAM13A worsens pulmonary hypertension by accelerating endothelial-to-mesenchymal transition in mice.

The paper determines the crystal structure of the BMPR2 kinase domain and analyzes how disease-associated mutations affect its stability and potential for therapeutic intervention.

The paper identifies two new risk genes for idiopathic pulmonary arterial hypertension through exome sequencing of 2,572 individuals.

This study identifies lncRNA-MEG3 as a contributor to hypoxic pulmonary hypertension by sequestering miR-328 and upregulating IGF1R in PASMCs.

This study identifies common genetic variants associated with pulmonary arterial hypertension risk and survival, focusing on HLA-DPA1/DPB1 and a SOX17 enhancer locus.

This study identifies novel rare genetic variants associated with heritable pulmonary arterial hypertension through whole genome sequencing of cases and controls.