In our study, in line with reports of Mavrogiannis [28] and Zhou [29], activated NLRP3 signaling was observed in both PAH rats and hypoxia-stimulated PMECs, which were markedly abolished by PHN-20 administration, implying the involvement of NLRP3 inhibition in PHN-20 function against PAH. The gene discussed is CPS1; the disease is pulmonary arterial hypertension.