As for ligands for RAGE, Greenway et al. reported that S100A4/Mts1 was expressed in smooth muscle cells of lesions showing neointimal formation and with increased intensity in vessels with an occlusive neointima and plexiform lesions of patients with pulmonary hypertension secondary to congenital heart disease [21]. The gene discussed is S100A4; the disease is pulmonary arterial hypertension.