Since CCN2 KO mice showed reduced arterial pressure, indicated by reduced RVSP, in the bleomycin model, we also investigated the impact of CCN2 gene deletion in another lung disease model: a severe model of PAH, which is induced by hypoxia and Sugen/SU5416, an inhibitor of vascular endothelial growth factor (VEGF). This evidence concerns the gene CCN2 and lung disorder.