BMPR2 and pulmonary arterial hypertension: These studies suggested that mutations in BMPR2 lead to mitochondrial dysfunction and hyperinsulinemia in IR-PAH progress and that this phenomenon is the result of different mechanisms, such as the occurrence of a lipid accumulation in skeletal muscle and the RV, and an impairment in mitochondrial homeostasis including the PA, with decreased mitochondrial respiration and DNA repair.