The familiar form of PAH has been linked with mutations in genes of the TGFβ signaling pathway (Liu et al., 2011; Reynolds et al., 2011; Frydman et al., 2012) and mutations in ion channels such as KCNK3 (K+ channel subfamily K member 3) (Ma et al., 2013) and ATP1A2 (NKA α2-subunit) (Montani et al., 2013). Here, TGFB1 is linked to pulmonary arterial hypertension.