We have also shown that in pathological conditions, dysfunctional ECs show altered intracellular trafficking and signaling of cell surface receptors such as the TGFβR1 (Bardita et al., 2015), implicated in the pathogenesis of PAH (Garcia-Rivas et al., 2017; Graf et al., 2018). This evidence concerns the gene TGFBR1 and pulmonary arterial hypertension.