Spontaneous Adult-Onset Pulmonary Arterial Hypertension Attributable to Increased Endothelial Oxidative Stress in a Murine Model of Hereditary Hemorrhagic Telangiectasia

Abstract

Adult Eng(+/-) mice acquire signs of pulmonary arterial hypertension that are attributable to uncoupled eNOS activity and increased (*)O(2)(-) production, which can be prevented by antioxidant treatment. Eng links transforming growth factor/bone morphogenetic protein receptors to the eNOS activation complex, and its reduction in the pulmonary vasculature leads to increased oxidative stress and pulmonary arterial hypertension.