Mitochondrial Dysfunction Dictates Energy Metabolism Reprogramming in Pulmonary Arterial Hypertension

We conclude that the MD secondary to the NFU1 insufficiency induces significant metabolic derangements, such as increased flux of fatty acids, glucose intolerance, and insulin insensitivity associated with classic metabolic disorders and recently implemented in the pathogenesis of PAH. The severe metabolic reprogramming revealed in PASMC correlates with progressive pulmonary vascular disease in NFU1 rats.