To further test if the Egln1Tie2Cre mouse model is an ideal preclinical PAH model, we determined the therapeutic responses of Egln1Tie2 mice to three FDA-approved PAH drugs which are vasodilators representing classical therapies of PAH by targeting three distinct signaling pathways, prostacyclin analogs (treprostinil), endothelin receptor antagonists (ambrisentan), and PDE5 inhibitor (sildenafil). The gene discussed is PDE5A; the disease is pulmonary arterial hypertension.