Dysregulated or aberrant TGFβ1 signaling is implicated in numerous pathological conditions including cancer, pulmonary hypertension, and a wide variety of organ-specific fibrotic diseases, including renal and idiopathic pulmonary fibrosis (IPF) [7], [8], [9], [10]. This evidence concerns the gene TGFB1 and pulmonary arterial hypertension.