Although we did not find any differences in RAGE (AGER) transcripts between heritable and non-heritable pediatric PAH, we did find heightened RAGE expression in vascular and inflammatory cells in the intima, media and adventitia of obliterated pulmonary arteries of both children with IPAH and PVOD and those with HPAH-BMPR2 +/− (Figure 6B–E). Here, AGER is linked to pulmonary arterial hypertension.