Quantitative analysis of RAGE (AGER) mRNA in children’s whole human lung tissues that were transplanted for either idiopathic PAH or pulmonary veno-occlusive disease (PVOD), vs. children with heritable PAH (HPAH, BMPR2 +/− mutation), revealed that the presence of a heterozygous BMPR2 gene mutation (HPAH, n = 4) did not influence the relative RAGE mRNA expression in HPAH-lungs vs. lungs from children that were transplanted for IPAH or PVOD/PAH (IPAH+PVOD n = 6; Figure 6A). Here, AGER is linked to heritable pulmonary arterial hypertension.