Some essential proteins, such as the redox-sensitive nuclear factor erythroid 2-related factor 2 (Nrf2), the AMP-activated protein kinase (AMPK), uncoupling protein 2 (UCP2), Nogo-B, dynamin-related protein (DRP) 1, mitofusin-2, and sarco-/endoplasmic reticulum calcium-ATPase (SERCA), are associated with mitochondrial metabolic functions in PAH [8–14]. Here, UCP2 is linked to pulmonary arterial hypertension.