Accruing evidence from a number of studies intensely suggests that Rho-A/Rho-kinase signaling plays a crucial role in the pathogenesis of various animal models of pulmonary hypertension and its activity is increased as well in the pulmonary artery smooth muscle cells of idiopathic PAH patients [79, 80]. The gene discussed is RHOA; the disease is pulmonary arterial hypertension.