EPAS1 and pulmonary arterial hypertension: Additionally, in multiple PAH models and patient samples, the glycolytic key enzyme PFKFB3 mediates macrophage inflammatory responses via regulation of the HIF-1a/HIF-2a signaling pathway, and its inhibitor 3PO reduces pulmonary macrophage numbers and pro-inflammatory cytokine secretion, effectively ameliorating PAH symptoms (82).