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Perturbations of metabolic pathways are an overarching theme spanning numerous pathological conditions and diseases, including pulmonary hypertension (PH). The majority of metabolic disturbances in PH can be linked with underlying mitochondrial dysfunction (1). Pulmonary artery cells from PH patients have higher glucose uptake, but lower oxidative glucose metabolism in the mitochondria (2-4). Similar to altered glucose handling, PH patients display higher lipid accumulation in the heart (5) and lower mitochondrial fatty acid oxidation in the pulmonary artery cells (4). Although metabolic disturbances are unlikely to be the causative factor in the development of PH, they give diseased cells a competitive growth and survival advantage such as the reported increase in glutaminolysis (6) and one carbon-glycine metabolism in PH patients (7). Ultimately, the mitochondrial dysfunction leads to more inefficient ATP …