Notably, glucose uptake is significantly upregulated in both the lungs and ECs of patients with idiopathic PAH (IPAH) and is accompanied by declines in EC mitochondrial density and higher rates of EC proliferation (Tuder et al., 2001; Fijalkowska et al., 2010; Cao et al., 2019), while heterozygous PFKFB3 deficiencies and the administration of a PFKFB3 inhibitor protected mice and rats, respectively, from hypoxia-induced PH (Cao et al., 2019). Here, PFKFB3 is linked to idiopathic pulmonary arterial hypertension.