Mice with specific deletion of BMPR2 in endothelial cells develop hypoxia-induced PAH, with decreased lung tumor protein p53 (EC p53), peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC1α), and transcription factor A (TFAM) regulators of mitochondrial biogenesis and DNA repair, leading to mitochondrial dysfunction (55). Here, PPARGC1A is linked to pulmonary arterial hypertension.