SOD2 and pulmonary arterial hypertension: This link between carnitine homeostasis, SOD2, and mitochondrial dysfunction may play an important role in the development of pulmonary hypertension as we have shown early derangements in SOD2 in our lamb model of pulmonary hypertension [30] while a recent study found that SOD2 expression and activity was decreased in PAEC isolated from patients with idiopathic pulmonary arterial hypertension (IPAH) [31].