Collectively, the dysregulation of these signaling pathways in PAH creates a state of pseudohypoxia, wherein cells of the pulmonary vasculature, despite normoxic conditions, manifest activation of HIF-1α and an acquired channelopathy which promotes pulmonary vascular constriction and remodeling typical of that seen after chronic hypoxia exposure. The gene discussed is HIF1A; the disease is pulmonary arterial hypertension.