This assumption is based on the fact that A2AAR KO mice exhibit structural and functional abnormalities similar to PAH (Xu et al., 2011; Shang et al., 2015) and overexpress RhoA and ROCK proteins, which are known to be involved in pulmonary vascular remodelling and PAH pathophysiology (Table 1) (Shang et al., 2015). Here, RHOA is linked to pulmonary arterial hypertension.